Respiratory Diseases

C H A P T E R
4
Respiratory Diseases
CARON M. HONG, MD, MSc n
RAFAEL CARTAGENA, MD n
ANTHONY N. PASSANNANTE, MD n
PETER ROCK, MD, MBA, FCCM n
KEY POINTS
Diseases of the Pulmonary Circulation
Pulmonary Arteriovenous Fistulas n Pulmonary arteriovenous fistulas have congenital and
Wegener's Granulomatosis hereditary etiology, and patients are at risk for life-threatening
Lymphomatoid Granulomatosis rupture requiring surgery.
Churg-Strauss Syndrome n Wegener's granulomatosis can affect any organ system,
Primary Pulmonary Hypertension although renal and pulmonary involvement is most com-
Obstructive Disease mon; men ages 40 to 50 are at increased risk.
Cystic Fibrosis n Lymphomatoid granulomatosis affects cardiopulmonary,
Infiltrative and Interstitial Diseases neurologic, and myeloproliferative systems; may result
Bronchiolitis Obliterans Organizing Pneumonia from opportunistic infection, and frequently progresses
Idiopathic Pulmonary Hemosiderosis to lymphoma; men age 50 to 60 are at increased risk.
Chronic Eosinophilic Pneumonia Spontaneous remission occurs in some cases; mortality is
Goodpasture's Syndrome 60% to 90% at 5 years.
Pulmonary Alveolar Proteinosis n Churg-Strauss syndrome is usually associated with long-
Sarcoidosis standing asthma, with men and women affected equally,
Systemic Lupus Erythematosus and can affect any organ system; major cause of death is
Idiopathic Pulmonary Fibrosis cardiac related.
Acute Respiratory Distress Syndrome
n Primary pulmonary hypertension is a diagnosis of exclu-
Pulmonary Histiocytosis X
sion; women are affected twice as likely as men; right-to-
Lymphangioleiomyomatosis
left shunt may occur in 30%, secondary to patent foramen
Arthritic Diseases Creating Upper Airway and
Respiratory Problems
ovale; hypoxia with resultant heart failure is typical cause
Ankylosing Spondylitis
of death.
Kyphosis and Scoliosis n Cystic fibrosis is an autosomal recessive disease, eventually
Drug-Induced Lung Injury fatal, with increased risk for airway obstruction, fluctuating
Bleomycin Toxicity pulmonary function, and chronic hypoxia; risk for sponta-
Infectious Diseases neous pneumothorax is 20%.
Influenza A (H1N1) n Bronchiolitis obliterans organizing pneumonia is a pulmo-
Severe Acute Respiratory Syndrome nary obstructive disease that may be reversible and usually
Echinococcal Disease of Lung resolves spontaneously.
Conclusion n Idiopathic pulmonary hemosiderosis is associated with
autoimmune disorders; patients have recurrent hemorrhage,
137
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138 ANESTHESIA AND UNCOMMON DISEASES
ulmonary fibrosis, restrictive lung disease, and pulmonary

p n Severe acute respiratory syndrome (SARS) is highly infec-
hypertension, with some cases of spontaneous remission. tious, transmitted by coronavirus with human-to-human
n Chronic eosinophilic pneumonia may be preceded by exposure via droplets or surfaces, and may progress to
adult-onset asthma; women are at increased risk; prognosis ARDS.
is good. n Echinococcal disease of lung is from canine tapeworm,
n Goodpasture's syndrome is a genetic autoimmune disorder transmitted by eggs from feces; rupture of cyst may result in
involving the pulmonary and renal systems. anaphylactic reaction or spread of disease to other organs;
n Pulmonary alveolar proteinosis, a lipoprotein-rich accu- children are at increased risk. No transthoracic needle aspi-
mulation in alveoli, has three forms: congenital, decreased ration is done; surgery is only option.
alveolar macrophage activity, and idiopathic; some cases of
spontaneous remission occur.
n Sarcoidosis may affect any organ system; African American, A thorough knowledge of pulmonary anatomy and physiol-
northern European, and females are at greater risk; many ogy is essential to the practicing anesthesiologist, who should
patients are asymptomatic. be familiar with common clinical conditions such as chronic
n Systemic lupus erythematosus may affect any organ system; obstructive lung disease (COPD) and asthma. This chapter
women of childbearing age are at increased risk. presents a comprehensive review of less common pulmonary
n Idiopathic pulmonary fibrosis is a rare interstitial lung dis- conditions, organized anatomically (pulmonary vasculature,
ease, with smokers at increased risk for pulmonary malig- airways, pulmonary interstitium), and conditions extrinsic
nancy; survival is usually 2 to 3 years from diagnosis; no to the lungs that affect pulmonary function, such as severe
effective treatment exists, with lung transplant the only arthritic disorders. Drug-induced lung injury is also discussed,
therapeutic option. followed by rare infectious pulmonary diseases, including
n Acute respiratory distress syndrome (ARDS) is associated influenza A (H1N1), severe acute respiratory syndrome, and
with underlying critical illness or injury, developing acutely echinococcal disease of the lung.
in 1 to 2 days; mortality is 25% to 35%. Many of these conditions are severe, and some are diffi-
n Pulmonary histiocytosis X is an interstitial lung disease cult to diagnose. Patients with pulmonary disease may present
associated with cigarette smoking and an unpredictable with varied symptoms, including productive or nonproductive
course; some spontaneous remission occurs. cough, fever, shortness of breath, chest pain, and decreased exer-
n Lymphangioleiomyomatosis involves progressive dete- cise tolerance. In most circumstances, patients who have these
rioration of lung function, associated with tuberous conditions will already be under the care of an internist or pul-
sclerosis and exacerbated by pregnancy, with women monary specialist. The patient evaluation necessary to arrive at
at increased risk; possible spontaneous pneumothorax an accurate diagnosis often is comprehensive, including detailed
and chylothorax; death usually results from respiratory history and physical examination; chest radiograph; pulmonary
failure. function tests (PFTs), including spirometry, diffusing capacity,
n Ankylosing spondylitis is a genetic inflammatory process and lung volume determination; and p erhaps arterial blood
resulting in fusion of axial skeleton and spinal deformities, gas (ABG) analysis. For some conditions, bronchoscopy and
with men at increased risk; radiologic bamboo spine, sacral to biopsy may be performed, and others require echocardiography
cervical progression, and restrictive lung disease with high reli- or cardiac catheterization for diagnostic certainty. For urgent or
ance on diaphragm; extraskeletal manifestations may occur. emergent surgery, the gravity of the clinical situation often pre-
n Kyphosis (exaggerated anterior flexion) and scoliosis (lat- cludes additional diagnostic assessment. For elective surgery,
eral rotational deformity) are spinal/rib cage deformities preoperative evaluation should include a review of these diag-
with idiopathic, congenital, or neuromuscular etiology; nostic studies and a determination as to whether the patient's
corrective surgery done if Cobb thoracic angle >50% lum- clinical condition has changed substantially. If a diagnosis has
bar angle >40%. already been established, there is no evidence to suggest that
n Bleomycin is an antineoplastic antibiotic used in combina- additional pulmonary testing will improve pulmonary outcomes
tion chemotherapy, with no myelosuppressive effect; toxic- after surgery. Spirometry and lung volume determination are the
ity can cause life-threatening pulmonary fibrosis. “gold standards” for the presence or absence of pulmonary dis-
n Influenza A is highly infectious, presenting with flulike ease, but are poor predictors of patients who will develop a pul-
symptoms and possible progression to ARDS; human- monary complication after surgery.1 If a diagnosis has not been
to-human exposure is through droplets or contaminated established in a patient who has symptoms consistent with one
surfaces, with high risk for infants, children, pregnancy, of these respiratory diseases, pulmonary consultation should be
chronically ill, or renal replacement therapy patients. obtained preoperatively as the patient's pulmonary disorder may
No prophylactic treatment exists; treat patients with be more urgent than an elective surgical procedure.
high index of suspicion without definitive testing; rRT- Unfortunately, pulmonary complications are common after
PCR and viral cultures are sensitive for pandemic H1N1 many surgical procedures, particularly those involving the
strain. upper abdomen or thorax, possibly more likely than c ardiac
Chapter 4 Respiratory Diseases 139
complications.2–5 Pre-existing lung disease, smoking, con-

gestive heart failure, American Society of Anesthesiologists BOX 4-2 n PULMONARY ARTERIOVENOUS FISTULAS:
ETIOLOGY
(ASA) classification, obesity, obstructive sleep apnea, anes-
thetic time in excess of 180 minutes, and advanced age are also Congenital
risk factors for pulmonary complications.4–9 There is no stan- Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome)
Chest trauma
dard definition of exactly what constitutes a pulmonary com-
Cavopulmonary shunting*
plication, but the most important complications are those that Hepatic cirrhosis
cause significant morbidity (e.g., postoperative pneumonia) Pulmonary hypertension
and postoperative respiratory failure. Because all the disorders
discussed in this chapter constitute pre-existing lung disease, *First stage of a Fontan repair for single ventricle physiology, generally
patients with these disorders who come to the operating room performed at 4 to 6 months of age. A cavopulmonary shunt is constructed
are at increased risk of postoperative pulmonary complica- and directs superior vena caval blood flow to the confluent pulmonary
arteries.
tions. Effective preoperative and intraoperative treatments are
discussed with the individual diseases. In the postoperative
period, aggressive treatment with mechanical measures such as
incentive spirometry can reduce pulmonary complications.10,11 middle-aged women but sometimes diagnosed in early child-
Other intraoperative interventions, such as laparoscopic sur- hood, are more likely to have multiple fistulas and more severe
gery, nasogastric tube decompression, and shorter-acting neu- symptoms.14
romuscular blockade, may also be beneficial.12,13 Patients with pulmonary AV fistula are at risk for rup-
ture, resulting in potentially life-threatening hemothorax
and hemoptysis. Thrombus formation within the fistula
DISEASES OF THE PULMONARY may also occur, with potential embolization of clot to the
CIRCULATION brain, resulting in stroke or seizures. Embolization of other
organ systems is also possible. If the thrombus becomes
Pulmonary Arteriovenous Fistulas
infected, septic emboli and potential abscess formation
Pulmonary arteriovenous (AV) fistulas are abnormal commu- may result.
nications between the arterial and venous pulmonary circula- Surgical intervention in the management of pulmonary
tion that result in shunting of blood from right to left without AV fistulas becomes necessary when the patient develops
traversing the pulmonary capillary network. This shunt results more pronounced cardiac symptoms, significant respiratory
in a decreased fraction of the pulmonary circulation partici- symptoms, room-air desaturation, or complications such as
pating in gas exchange, mixing of oxygenated and deoxygen- emboli with central nervous system (CNS) manifestations.
ated blood, and a consequent reduction in arterial oxygen Surgical preoperative evaluation requires chest computed
tension (Pao2). Many patients with pulmonary AV fistulas are tomographic angiography (CTA) or pulmonary arteriogra-
asymptomatic, but some may have associated signs and pos- phy to localize the lesion. Pulmonary lobectomy, segmentec-
sible symptoms consistent with chronic hypoxemia (Box 4-1). tomy, or wedge resection using thoracotomy or video-assisted
Known causes of pulmonary AV fistula formation include thoracoscopic surgery (VATS) are the most common proce-
congenital malformations (Box 4-2). Patients with heredi- dures. Embolization procedures are becoming the preferred
tary hemorrhagic telangiectasia (Osler-Weber-Rendu syn- treatment for the majority of patients because embolization
drome), an autosomal dominant syndrome most often seen in is less invasive, is easily repeated, and may be an adjuvant to
decrease bleeding and other complications during definitive
surgical resection.15,16
Anesthetic evaluation focuses on the degree of shunt and
BOX 4-1 n PULMONARY ARTERIOVENOUS FISTULA:
SIGNS AND SYMPTOMS hypoxemia, using ABG analysis. Review of the pulmonary
angiogram will reveal the size of the lesion and whether mul-
Shortness of breath tiple fistulas are present. A significant fistula in the nonop-
Dyspnea with exertion
erative lung may compromise arterial oxygenation if one-lung
Bloody sputum
Cyanosis ventilation is required for surgical exposure. Efforts to mini-
Clubbing mize flow through a pulmonary AV fistula involve avoiding
Chest pain both increased pulmonary vascular resistance (PVR) and ele-
Palpitations vated levels of positive end-expiratory pressure (PEEP), both
Bruit
of which will increase flow through the low-resistance fistula.
Low arterial oxygen saturation
Polycythemia Intraoperative management frequently requires one-lung
Anemia ventilation to optimize surgical exposure. A double-lumen
Abnormal vasculature or nodules on chest radiograph endotracheal tube (ETT) provides the added benefit of iso-
lating the nonoperative lung and airways from any bleeding,
which may occur during a potentially bloody resection. The Wegener's Granulomatosis
risk of significant bleeding is decreased if the lesion has been
Wegener's granulomatosis (WG) is a rare disorder charac-
embolized before resection. Large-bore intravenous (IV)
terized by necrotizing giant cell granulomatosis of the upper
access is recommended in the event significant hemorrhage
respiratory tract and lung, widespread necrotizing vasculitis,
occurs. An arterial catheter is also indicated to monitor oxy-
and focal glomerulonephritis. WG may also affect the cardio-
genation and guide resuscitative efforts. As mentioned, an
vascular, neurologic, and gastrointestinal systems.17 Although
important anesthetic goal is to minimize flow through the
the etiology of WG is unknown, an autoimmune disorder is
pulmonary AV fistula. AV fistulas do not have capillary beds
suspected. A typical patient is in the fourth or fifth decade, and
and have lower resistance to blood flow than normal pulmo-
men are twice as likely to have WG as women. Antineutrophil
nary vasculature. It is important to avoid a general increase
cytoplasmic autoantibody (ANCA) is a serologic marker
in PVR because this will increase flow through the AV fis-
that can help confirm the diagnosis.18 Staphylococcus aureus
tula. Similarly, minimizing the use of PEEP will minimize
has been implicated as an exacerbating cofactor.18 Symptoms
increases in PVR and help minimize blood flow through
associated with WG are vague, and diagnosis can be elusive
the fistula. Because of the risk of paradoxical emboli pass-
(Box 4-4). Biopsy of a lesion is necessary to make the diagnosis.
ing through the fistula, extra caution must be taken to avoid
If the disease progresses, significant respiratory and renal
injection of any air or particulate material into the venous
compromise can occur, as well as hearing and vision loss
system, because such debris may bypass the pulmonary cap-
(Box 4-5). Cardiac involvement is uncommon, although peri-
illary bed and gain access to systemic arteries, where end-
carditis, coronary arteritis, valvular involvement, and left ven-
organ embolization can occur (Box 4-3).
tricular hypertrophy have been reported. Current therapy for
Preoperative evaluation should include assessment of neu-
WG is often based on disease severity but usually includes
rologic function to rule out prior embolic stroke. Postoperative
cyclophosphamide, corticosteroids, methotrexate, or azathio-
evaluation should include a neurologic check as well, to look
prine and yields very good results, with long-term remission
for perioperative CNS embolization.
occurring in the majority of patients. Recent studies have dem-
onstrated possible advantages of antistaphylococcal antibiotics
BOX 4-3 n ANESTHESIA CONCERNS FOR PATIENTS and T-cell inhibitors (leflunomide).18 Preoperative assessment
WITH PULMONARY DISEASE is directed toward evaluating potential complications of WG,
most often renal and pulmonary insufficiency. Blood urea
Pulmonary Arteriovenous Fistula
Assess for degree of shunt and hypoxemia.
Avoid increases in pulmonary vascular resistance.
BOX 4-4 n WEGENER'S GRANULOMATOSIS: COMMON
Avoid elevated positive end-expiratory pressures.
SIGNS AND SYMPTOMS
Extra care is needed to prevent unintentional intravenous air injection
or any condition that would result in a venous air embolism. Hematuria
Wegener's Granulomatosis Shortness of breath
Assess for specific organ system involvement (renal and pulmonary Wheezing
insufficiency). Hemoptysis
Avoid nasal manipulation (nasal intubation). Bloody sputum
Assess for risk of difficult airway (subglottic/tracheal stenosis). Cough
Chest pain or pleuritis
Lymphomatoid Granulomatosis Sinusitis
Assess extent of organ system involvement (obstructive or restrictive Ulcers or lesions around nose
lung disease, cardiomyopathy, neuropathy, myelosuppression). Weight loss
Possible adrenal suppression from long-term steroid treatment. Weakness
Churg-Strauss Syndrome Fever
Assess level of organ system involvement (PFTs, chest radiograph, Joint pain
ECG, echocardiogram).
Minimize airway manipulation secondary to airway hyperreactivity.
May need stress-dose perioperative steroids.
BOX 4-5 n WEGENER'S GRANULOMATOSIS:
Primary Pulmonary Hypertension COMPLICATIONS
Consider increased perioperative morbidity and mortality.
Complete cardiopulmonary workup is needed for all procedures (ECG, Chronic renal insufficiency or renal failure
echocardiography, chest radiograph, ABGs). Hearing loss
Spinal anesthesia is not recommended. Subglottic/tracheal stenosis
Maintain cardiac output and systemic vascular resistance. Pulmonary insufficiency
Minimize increases in pulmonary vascular resistance. Functional nasal deformities
Consider invasive monitoring intraoperatively. Ocular abnormalities
Restrict nitrous oxide or ketamine use. Vision loss
ABGs, Arterial blood gases; ECG, electrocardiogram; PFTs, pulmonary Ulcerative keratitis
function tests. Orbital pseudotumor
nitrogen (BUN) and creatinine levels will p rovide adequate

insight into the patient's renal function. A pulmonary flow- BOX 4-6 n LYMPHOMATOID GRANULOMATOSIS:
CLINICAL MANIFESTATIONS
volume loop may be indicated if the patient is suspected of
having tracheal stenosis and, by providing information about Hemoptysis
the dynamic changes in tracheal caliber, can supplement static Cough
Dyspnea
radiographic images. WG may cause either obstructive or
Chest pain
restrictive lung disease; the latter can be severe. Spirometry Pneumothorax
and other PFTs such as formal lung volume measurements can Pleural effusions
help determine the severity of such disease. Bronchoscopy and Atelectasis
neck/chest CT may be necessary to evaluate subglottic stenosis Fever and weight loss
Hepatomegaly
and suggest which EET size can be placed safely.
Erythema
Several aspects of WG may complicate management of the Mononeuritis multiplex
patient's airway. A significant amount of granulation tissue is Peripheral sensory neuropathy
likely to be present in and around the nose and nasopharynx.
Insertion of a nasotracheal tube or nasal airway may be impos-
sible, or traumatic with hemorrhage, and is best avoided. this clinical p
icture. LYG is frequently fatal, with 60% to 90%
Additionally, lesions on the epiglottis or oropharynx may mortality at 5 years, although a small number of patients may
inhibit direct laryngoscopy, despite a normal airway exami- undergo spontaneous recovery and complete remission. The
nation. Once the vocal cords have been visualized, the ETT cause of death is usually related to extensive destruction of the
may be difficult to place because of subglottic stenosis and lungs and resulting respiratory failure.20,21
may require multiple l aryngoscopies. If the patient is receiving Corticosteroids and cyclophosphamide are the treat-
corticosteroids at the time of surgery, stress dosing should be ment of choice, resulting in relief of symptoms such as fever,
considered (see Box 4-3). cough, chest pain, weight loss, and sinusitis. If not diagnosed
In view of these concerns, it is best to proceed with a con- in the premalignant phase, and if the disease has progressed
servative plan for managing the airway in WG patients, with to lymphoma, chemotherapy is necessary. The combina-
immediate availability of difficult airway equipment, mul- tion of cyclophosphamide, doxorubicin (hydroxydaunomy-
tiple sizes of ETTs, a videolaryngoscope or fiberoptic bron- cin), vincristine (Oncovin), and prednisone (CHOP) is often
choscope, and the means to obtain a surgical airway, as a last used. Radiation therapy may be indicated for localized dis-
resort. If the patient has significant tracheal or bronchial ste- ease. More recently, immunomodulation with interferon
nosis, care should be taken to prevent air trapping and auto- alfa-2b and autologous stem cell transplantation have played
PEEP by allowing sufficient time for exhalation if the tracheal a role in treatment.20,21
lesion is below the ETT. (See also Chapter 1.) In preparation for anesthesia, evaluation of the patient's
pulmonary function is the primary concern with LYG. Chest
radiography may reveal bilateral nodules, cavitations, pleural
Lymphomatoid Granulomatosis
effusions, or pneumothorax. In the presence of advanced
Lymphomatoid granulomatosis (LYG), also known as angiocentric disease, ABG analysis and spirometry help define the extent
lymphoma, is a rare lymphoproliferative disease that is angiode- of the patient's respiratory compromise and parenchymal
structive and frequently progresses to lymphoma. LYG mimics destruction. A thorough preoperative n eurologic evaluation
WG clinically and radiographically, although recent advances have is advised because of the high incidence of peripheral neu-
identified LYG as a malignant B-cell lymphoma associated with ropathy. Toxicities related to any chemotherapeutic agents the
immunosuppression and Epstein-Barr virus (EBV). Diagnosis patient may have received should also be considered. Toxicity
requires histologic evaluation of a biopsy specimen. LYG was related to the CHOP protocol includes peripheral neuropathy,
recently categorized as a lymphoma, although if diagnosed early cardiomyopathy, and myelosuppression.
(grade I angiocentric immunoproliferative lesions), it is consid-
ered benign, although premalignant.19 Typically, it presents in the ANESTHETIC MANAGEMENT
fifth or sixth decade of life, affecting men twice as often as women. When planning an anesthetic for a patient with LYG, the pres-
The etiology of LYG is unknown, although its incidence in pop- ence of or potential for peripheral neuropathy may deter the
ulations with immune dysfunction, such as human immunode- anesthesiologist from using regional techniques, because of
ficiency virus (HIV) patients and organ transplant recipients, is concern that subsequent neurologic dysfunction will be attrib-
significantly increased compared with the general population. uted to the regional anesthesia. However, the choice of anes-
Speculation that LYG resulted from an opportunistic infection has thetic must be based on a consideration of risks and benefits,
been confirmed through laboratory investigation. and there is no evidence that regional anesthesia worsens LYG.
The disease process primarily involves the lungs, although Respiratory compromise increases the risk of hypoxia under
the skin, kidneys, and CNS can also be affected. Signs and general anesthesia, or if the patient hypoventilates secondary
symptoms of LYG include an increased risk of pneumo- to sedating agents used for premedication or for monitored
nia (Box 4-6). Unlike WG, glomerulonephritis is not part of anesthesia care.
If general anesthesia is chosen, the potential for postopera- Preoperative assessment should include a chest radiograph
tive intubation and respiratory support should be addressed and PFTs. Chest radiography may reveal multiple small pulmo-
with the patient. The need for postoperative mechanical ven- nary nodules or diffuse interstitial disease. Pleural effusions are
tilation is more likely in patients who have advanced disease noted in up to 30% of CSS patients. Spirometry typically demon-
with extensive destruction of lung tissue, pleural effusions, or strates an obstructive pattern, although restrictive disease may
pneumothorax. There is no clear answer to which anesthetic also occur. A decrease in diffusion capacity may be observed
technique is superior, and the approach should be tailored to from a loss of alveolar capillary surface area. Intraoperative
the individual patient's comorbidities and the surgical proce- management should include universal asthmatic principles
dure. Long-term corticosteroid therapy in this population may to minimize airway reactivity. If possible, avoidance of air-
result in adrenal suppression, and stress doses of corticoste- way instrumentation and positive-pressure ventilation (PPV)
roids should be considered (see Box 4-3). is desirable. A prolonged expiratory phase may be needed in
patients with more advanced obstructive disease if PPV is used,
and preoperative spirometry will provide guidance in this area.
Churg-Strauss Syndrome
Nonselective beta-adrenergic blockers should be avoided, if
Churg-Strauss syndrome (CSS), also known as allergic granu- possible, because of the risk of bronchospasm and exacerba-
lomatosis, is a rare systemic vasculitis that may affect multiple tion of CHF. If needed for control of ischemic heart disease,
organ systems, particularly the lungs. Diagnosis requires the selective β1-adrenergic agents, preferably short acting, should
presence of at least four of six criteria: bronchospasm, eosin- be used. Perioperative corticosteroids should be considered
ophil count greater than 10%, neuropathy (poly or mono), because of the risk of adrenal suppression from long-term cor-
nonfixed pulmonary infiltrates, paranasal sinus abnormali- ticosteroid therapy (see Box 4-3).
ties, and extravascular eosinophils22 (Box 4-7). Patients fre-
quently present in the fifth or sixth decade and may have a
Primary Pulmonary Hypertension
long-standing history of asthma. Both genders are affected
equally. Cardiac involvement occurs later in the course and Primary pulmonary hypertension (PPH) is an idiopathic dis-
is the major cause of death. CNS manifestations such as cere- ease and is a diagnosis of exclusion. The prevalence of PPH
bral infarcts, subarachnoid hemorrhage, and optic neuritis is thought to be approximately 1:1 million, with women
are common. being twice as likely as men to present with the disease. Some
Corticosteroids generally result in dramatic improvement cases appear to be genetically linked.24 Overall, PPH is more
or resolution of CSS symptoms. Cytotoxic therapy should be severe and aggressive than secondary pulmonary hyperten-
initiated based on the severity of disease. Patients resistant sion. Vascular remodeling, an alteration in pulmonary vascu-
to corticosteroids may respond to interferon-α treatment.23 lar tone, and a loss of cross-sectional pulmonary arterial area
Elective surgery should be postponed if management of bron- are responsible for the increase in PVR seen in this disease.
chospasm has not been optimized. Involvement of other organ Dyspnea is the most common presenting symptom, and syn-
systems may necessitate neurologic and renal evaluations. cope is a particularly poor prognostic sign (Box 4-8). Right-
Cardiac evaluation may require testing such as echocardiogra- to-left shunting may occur in the 30% of patients with a patent
phy to assess myocardial function if the patient has congestive foramen ovale (PFO). Death typically results from hypoxia,
heart failure (CHF) or endocarditis. a further increase in pulmonary artery pressure (PAP), and
eventually right ventricular (RV) failure.25
Historically, treatment for PPH relied on oxygen and cal-
BOX 4-7 n CHURG-STRAUSS SYNDROME: CLINICAL cium channel blockers in an effort to decrease PVR (Table 4-1).
MANIFESTATIONS In addition, warfarin (Coumadin) is used to reduce the risk of
thromboembolism resulting from the enhanced platelet activ-
Sinusitis
Nasal polyps
ity seen in PPH. Pulmonary embolism or primary pulmonary
Pulmonary infiltrates vascular thrombosis is poorly tolerated in this patient popula-
Diffuse interstitial lung disease (rare) tion. Diuretics and digoxin are also employed when RV failure
Hemoptysis ensues. More recently, prostaglandins (PGI2, PGE1; alprostadil)
Pleural effusions
Cutaneous nodules and rashes
Hypertension
Glomerulonephritis BOX 4-8 n PRIMARY PULMONARY HYPERTENSION:
Coronary vasculitis SIGNS/SYMPTOMS
Endocarditis
Congestive heart failure Dyspnea
Peripheral neuropathy Fatigue
Mononeuritis multiplex Syncope or presyncope
Cerebral infarct Angina
Subarachnoid hemorrhage Peripheral edema and other signs of right-sided heart failure
Optic neuritis Cyanosis
TABLE 4-1 n Current Therapies for Primary Pulmonary Hypertension
Therapy Advantages Disadvantages
Nitric oxide (NO) Pulmonary circulation with selective Possible formation of toxic byproducts; prolonged
vasodilation; increased Pao2 bleeding times; expensive
Prostaglandins (epoprostenol, Potent vasodilation; inhibits platelet Not selective for pulmonary circulation; systemic
treprostinil, iloprost) aggregation and smooth muscle cell hypotension; headaches; expensive; requires
proliferation continuous infusion or inhalation
Phosphodiesterase-5 inhibitors Possible synergy with NO therapy inhibitors —

(dipyridamole, sildenafil)
Endothelin receptor antagonist FDA approval Limited data available

(Bosentan)
Calcium channel blockers High efficacy; inexpensive Less effective in severe cases; negative inotropic
effects can worsen right ventricular failure
Oxygen Directly reduces pulmonary vascular None

resistance in cases of hypoxia
Warfarin (Coumadin) Improved long-term survival; decreases Increased bleeding risk

risk of intrapulmonary thrombosis
Magnesium Vasodilation through blockage of Ca2+ Risk of magnesium toxicity: weakness, sedation,
channels; enhance NO synthase activity; ECG changes
releases prostaglandin I
ECG, Electrocardiographic; Pao2, arterial oxygen tension (partial pressure).
and nitric oxide (NO), alone or in combination, have been

TABLE 4-2 n Preoperative Studies to Assess Pulmonary
used to induce pulmonary vasodilation, with minimal sys- Hypertension
temic effects.24,26 Currently, prostacyclins must be delivered by
continuous IV infusion because of their short half-life. NO is Study Possible Significant Findings
delivered by inhalation and requires a tank and delivery system.
Arterial blood gas Level of hypoxemia and acidosis; assess
Phosphodiesterase-5 inhibitors such as sildenafil and dipyri- analysis relative value of supplemental oxygen.
damole potentiate the NO-induced pulmonary vasodilation
and can be used separately or in combination.24 Unfortunately, Chest radiography Enlarged pulmonary arterial root;
enlarged right side of heart
cost and unwieldy delivery systems have limited the use of
these therapies to the short term or the most severe cases. New Electrocardiography Dysrhythmias; signs of right-sided
approaches to delivering PGI2 are under development, includ- heart strain
ing the inhaled, subcutaneous, and oral routes. A newer agent, Echocardiography Assess right ventricular function and
bosentan, an oral endothelin receptor antagonist thought to hypertrophy, valvular dysfunction and
inhibit smooth muscle vasoconstriction and proliferation, is right atrial enlargement, and patency
now approved by the U.S. Food and Drug Administration (FDA) of foramen ovale; estimate pulmonary
artery pressure.
to treat PPH.24,27 Adjunctive therapy with bosentan has demon-
strated promise when combined with prostacyclin therapy.26,27
Preoperative studies focus on the severity of PPH, degree e stimated by Doppler techniques. A more accurate but much
of hypoxia, and resulting effects on the heart (Table 4-2). ABG more invasive method of measuring pulmonary pressures,
analysis elucidates the level of hypoxia and acidemia, both of gauging response to therapies, and detecting a PFO is right-
which exacerbate pulmonary hypertension. A chest radiograph sided heart catheterization. This procedure should be con-
may reveal enlarged main pulmonary arteries or an enlarged sidered only if other studies have not provided an adequate
heart caused by RV hypertrophy or right atrial dilation. An assessment of disease severity and is not typically needed for
electrocardiogram (ECG) may also reveal changes consistent preanesthetic evaluation. The patient treated with digoxin
with pulmonary hypertension (e.g., right atrial enlargement), should have serum potassium and digoxin levels measured.
as well as the presence of abnormal cardiac rhythm (e.g., atrial
fibrillation). Sinus rhythm is essential to adequate RV filling. ANESTHETIC MANAGEMENT
Preoperative echocardiography is helpful in determining the The increased perioperative morbidity and mortality of this
extent of RV hypertrophy and function, right atrial enlarge- disease must be considered when preparing to deliver an anes-
ment, pulmonic or tricuspid valve dysfunction, and patency thetic to the PPH patient, and not assume the risk of peri-
of the foramen ovale. Pulmonary systolic pressures may be operative complications is low with a “minor” procedure
(see Box 4-3). Regional anesthetic techniques do not preclude conditions. It results in a significant reduction in life expec-
the need for possible invasive monitoring and vasoactive ther- tancy and quality of life. The responsible gene is found on the
apy. Each patient's needs should be considered individually. long arm of chromosome 7 and codes for a protein known as
All medications being used to treat the patient's PPH and cystic fibrosis transmembrane (conductance) regulator (CFTR),
resulting right-sided heart failure should be continued in the which functions as a chloride channel. This defect decreases
perioperative period. Warfarin should be discontinued and the water content of various secretions throughout the body,
replaced with a heparin infusion preoperatively. The risk of a resulting in increased viscosity. Diagnosis is based on sweat
thromboembolic event and a possible right-to-left shunt jus- chloride measurements, genetic testing for the CFTR gene, and
tify a preoperative hospital admission to administer heparin. clinical symptoms.28 CF is a universally fatal disease, although
Sedation must be carefully titrated; oversedation may lead to advances in therapy have resulted in significant gains in qual-
hypoxia, whereas not adequately addressing a patient's anxiety ity of life and longevity. A wide variety of clinical manifesta-
may also increase PVR. tions are seen in CF patients (Table 4-3).
Intraoperative management of PPH patients should Pulmonary manifestations result from the inability to
emphasize maintenance of cardiac output and systemic blood clear thickened and inspissated mucus from the airways. This
pressure (BP) while minimizing further increases in PAP causes airway obstruction and impaired defense against bacte-
and the risk of RV failure. Invasive monitors, used selec- rial infection, which results in the majority of deaths related to
tively, including an arterial catheter, PAC, and transesopha- CF. Recurrent bacterial infections result in dilation of the con-
geal echocardiography (TEE), allow for sampling of arterial ducting airways, leading to bronchiectasis.29 Although CF is a
blood, pharmacologic manipulation of PAP and cardiac out- chronic progressive disease, the extent of current pulmonary
put, and detection of RV failure, while maintaining adequate infection fluctuates, creating significant daily variability in a
ventricular preload. patient's pulmonary function. Eventually, as the disease pro-
Many different anesthetic techniques have been used suc- gresses, there is destruction of parenchyma and conduction
cessfully in patients with PPH; regional, epidural, and gen- airways. Loss of pulmonary arterial vascular cross-sectional
eral approaches with controlled ventilation are all reasonable area results in pulmonary hypertension. Chronic hypoxemia
options. Spinal anesthesia may result in a significant reduc- also develops.
tion in systemic vascular resistance (SVR) and may precipi-
tate a drop in preload with no change in pulmonary vascular
pressures. This may result in inadequate coronary flow
to perfuse the right side of the heart, with consequent RV TABLE 4-3 n Cystic Fibrosis: Clinical Manifestations
ischemia and failure. Drugs typically used in the provision
Sign/Symptom Cause
of anesthesia are safe in patients with PPH. An exception
is nitrous oxide (N2O), which has been implicated in rais- Nasal sinusitis, polyps Abnormal mucus production and
ing PVR in several studies. Another exception is ketamine, secretion; chronic infection
which has sympathomimetic properties and may cause unin- Chronic bronchitis Hypersecretion of viscid mucus;
tended PVR increase. impaired host defenses
If PVR does increase, every effort must be made to avoid
Obstructive pulmonary Chronic pulmonary infections and
RV ischemia and possible RV failure. Helpful maneuvers disease airway plugging from excessive
include hyperventilation and maximizing Pao2 to decrease mucus secretion
PVR. Inhaled drugs such as NO (20-40 ppm), and prostacy-
Pneumothorax Rupture of subpleural blebs
clin (inhaled/IV) can selectively decrease PAP with minimal
through visceral pleura
decreases in systemic BP. Milrinone and amrinone are excel-
lent choices to decrease PVR and increase cardiac contrac- Failure to thrive Chronic infection; malabsorption
tility, although SVR will also be decreased. Dobutamine will Recurrent pancreatitis Obstruction of pancreatic ducts
increase contractility and may decrease PVR. To increase with viscous exocrine secretions
systolic BP and avoid RV ischemia, norepinephrine may
Gastroesophageal reflux Unknown
have a slight advantage over phenylephrine.27 Maintenance disease
of adequate intravascular volume and RV preload is also
important. Maldigestion Biochemically abnormal intestinal
mucins impair absorption of
specific nutrients; abnormal bile
secretion and absorption
OBSTRUCTIVE DISEASE
Fat-soluble vitamin Abnormal bile secretion and
Cystic Fibrosis deficiencies absorption
Cystic fibrosis (CF) is an autosomal recessive genetic disease Obstructive azoospermia Atretic or absent vas deferens
that affects chloride channels. With an incidence of 1 per 2000
Salt-loss syndromes Inability to create hypotonic sweat
to 4500 Caucasians, CF is one of the more common inherited
Patients with more advanced CF may develop spontaneous respiratory compromise, and only then under close obser-
pneumothorax. The etiology of pneumothorax is unknown vation with administration of supplemental oxygen to
but presumably involves rupture of subpleural blebs through minimize the risk of desaturation. All CF patients should
the visceral pleura. This becomes more likely in advanced be questioned regarding symptoms consistent with GERD.
disease. Over a lifetime, the incidence of pneumothorax may If present, appropriate premedications and aspiration
be as high as 20% in adult CF patients. Application of PPV precautions such as a rapid-sequence induction should be
can increase the risk of spontaneous pneumothorax. In the considered, although CF patients may desaturate rapidly
event of pneumothorax, surgical pleurodesis is the treat- when apneic.
ment of choice for CF patients who have a low anesthetic
risk; higher-risk patients frequently receive talc pleurode- ANESTHETIC MANAGEMENT
sis as a safer, yet less effective, alternative.30 Ventilation/ Choice of anesthetic technique will be primarily determined by
perfusion inequality results in hypoxemia. The chronic the scheduled procedure, although regional techniques offer
hypoxia seen in this population causes an increase in PVR some advantages. Avoidance of airway instrumentation will
and pulmonary hypertension. Loss of pulmonary arterial decrease the risk of bronchospasm and aspiration. Avoiding
vascular cross-sectional area also causes increased PVR PPV will decrease the incidence of perioperative pneumotho-
and pulmonary hypertension, which is exacerbated by rax formation. If a long-acting or continuous regional tech-
chronic hypoxemia. The severity of pulmonary hyperten- nique is chosen, postoperative opioid requirements will be
sion correlates with the severity of CF. Chronic pulmonary less. The risk of postoperative respiratory insufficiency may be
vasoconstriction (from hypoxia) results in a musculariza- less with regional anesthetic techniques, although this has not
tion of the pulmonary arterial vascular tree, which results been rigorously studied.
in cor pulmonale, although the initial enlargement of the The plan for general anesthesia should take into account
right v entricle is considered a beneficial adaptation to the the increased risk of aspiration (from GERD) and bron-
increased resistance to pulmonary blood flow. The only chospasm. The likelihood of chronic sinusitis and the pres-
medical therapy effective in treating pulmonary hyperten- ence of paranasal sinus polyps are reasons to avoid nasal
sion and improving RV performance in this population is instrumentation, if possible. A rapid-sequence induction
supplemental oxygen.31 Although lung transplantation has proceeded by nonparticulate antacids and H2 antagonists
been successful with a 2-year survival of greater than 50%, may help minimize the likelihood and consequences of
about 40% of patients do not survive awaiting the trans- pulmonary aspiration of gastric contents. However, use of
plant due to organ shortage.28 rapid-sequence induction may result in uncontrolled sys-
The primary gastrointestinal manifestation of CF is mal- temic and pulmonary hemodynamics, and its use must
absorption and steatorrhea caused by pancreatic dysfunction balance airway risks with the risk of cardiovascular insta-
from obstruction of pancreatic ducts with viscous exocrine bility. PPV is usually preferable to spontaneous ventilation
secretions, usually requiring pancreatic enzyme replacements in advanced cases of CF, because of the risk of respiratory
as well as multivitamins. Malnutrition and deficiencies of fat- fatigue and marginal tidal volumes. CF is an obstructive
soluble vitamins such as vitamin K can increase the patient's process, and prolonged expiratory times may be neces-
risk of bleeding if this issue is not addressed. Glucose intoler- sary, as well as humidification of inspired gases and min-
ance resulting from pancreatic dysfunction (impaired endo- imization of peak airway pressures to reduce the risk of
crine function) is also common and may require insulin barotrauma and pneumothorax. Low respiratory rates and
therapy. CF patients also have an increased incidence of gas- smaller-than-usual tidal volumes may be required. Nitrous
troesophageal reflux disease (GERD).32 oxide should be used with caution because of the increased
Preparation for anesthesia should focus on evaluation risk of pneumothorax formation with PPV, as well as the
of the CF patient's pulmonary status. Significant variation likely presence of multiple blebs. Meticulous attention
in symptoms and disease severity from increased respira- to pulmonary toilet and suctioning of secretions is also
tory secretions or infection can be seen in a patient from advisable (Box 4-9).
one day to the next. Surgery should be postponed, if pos-
sible, unless the patient is at a baseline level of health.
Preoperative testing should include a recent chest radio- BOX 4-9 n ANESTHESIA CONCERNS WITH CYSTIC
FIBROSIS PATIENTS
graph to diagnose pneumothorax, pneumonic processes,
or bullous disease. In one series of patients with CF, 16% Assess cardiopulmonary function.
had an asymptomatic pneumothorax. Thus, chest radiog- Aspiration precautions should be considered secondary to
raphy is essential in these patients.30 Coagulation studies association with gastroesophageal reflux disease.
Avoid airway instrumentation if possible.
such as prothrombin time and partial thromboplastin time
Avoid positive-pressure ventilation if possible.
can provide information regarding coagulopathy result- Consider regional techniques when applicable and appropriate.
ing from vitamin K deficiency or general malnutrition. Avoid nasal instrumentation.
Sedating premedications should be given only if absolutely May need to prolong expiratory times.
necessary, because of the risk of exacerbating pre-existing
INFILTRATIVE AND INTERSTITIAL DISEASES because of a decreased functional residual capacity (FRC). The
use of low levels of PEEP will improve FRC and assist in main-
Bronchiolitis Obliterans Organizing Pneumonia taining Pao2. Continuation of PEEP or continuous positive
Bronchiolitis obliterans organizing pneumonia (BOOP) is an airway pressure (CPAP) in the postoperative period may be
inflammatory lung disease of unknown etiology. It has been necessary to maintain functional residual capacity (Box 4-10).
associated with bone marrow transplantation, although there
is a very low incidence of BOOP in this population;33 it has not
Idiopathic Pulmonary Hemosiderosis
been conclusively determined to be more than an incidental
finding. BOOP results from the formation of granulation tis- Idiopathic pulmonary hemosiderosis (IPH) is a rare d isorder
sue, which obstructs the lumen of small airways and extends of unknown etiology characterized by diffuse alveolar hem-
into the alveoli. The formation of the granulation tissue is orrhage. A diagnosis of exclusion, IPH is primarily seen in
associated with connective tissue proliferation, fibrinous exu- infants and children. There is an association with cow's milk
dates, and inflammation of alveolar and airway walls. These hypersensitivity, celiac disease, autoimmune hemolytic ane-

changes yield a clinical picture that presents as a flulike illness mia, and several other autoimmune disorders, such as lupus,
with cough and dyspnea. BOOP shares many characteristics periarteritis nodosa, and WG (see previous WG section), which
of idiopathic pulmonary fibrosis, with the most significant suggests an immunologic basis for IPH, but no firm relationship
difference being the reversibility of the fibrinous changes in has been established. Clinically, IPH is similar to the immune-
BOOP as a result of the preservation of lung architecture.33 mediated alveolar hemorrhage seen in syndromes such as
Corticosteroids are often used, although some cases resolve Goodpasture's syndrome (see Goodpasture's syndrome s ection)
spontaneously. Typically, therapy lasts for 1 year, with resolu- and WG, although extrapulmonary involvement is not present
tion of symptoms by the end of the third month of treatment. as it is in these disorders. Hemoptysis, anemia, and pulmonary
Symptoms may recur, particularly if the course of corticoste- infiltrates on chest radiograph are the common presenting signs
roids is not completed. Other agents such as erythromycin and and symptoms. The clinical course of IPH is variable, with some
cyclophosphamide have been used, although their efficacy is reports of spontaneous remission. Other patients will die sud-
not well established. Patients who received cyclophosphamide denly of severe alveolar hemorrhage or more gradually from
are at risk of leukopenia and, more rarely, thrombocytopenia respiratory insufficiency within 3 years of initial presentation.
or anemia. As a result of recurrent hemorrhage, pulmonary fibrosis with
Radiologic evaluation is consistent with an organizing restrictive lung disease and eventually pulmonary hypertension
pneumonia with patchy consolidation in a diffuse peripheral and cor pulmonale will ensue (Table 4-4).
distribution. Effusions are a rare finding. Spirometry typically Corticosteroids are the cornerstone of therapy for IPH.
demonstrates a restrictive pattern, although it is possible to Although the long-term efficacy of corticosteroid therapy for
find an obstructive component. Decreased diffusion capacity IPH is unclear, it is still the best option currently available.
and an increased alveolar-arterial oxygen gradient are com- Long-term, if not lifelong, therapy is usually required, and
mon. Definitive diagnosis requires lung biopsy, typically per- complications arising from corticosteroid therapy are a con-
formed thoracoscopically. BOOP occurs in 25% to 50% of cern, which leads physicians to minimize doses. This increases
long-term survivors of lung transplants, and 10% of all lung the risk of recurrence. Treatments with plasmapheresis, aza-
transplant recipients, indicating a poor prognosis.34 It is a thioprine, and cyclophosphamide have been attempted with
manifestation of chronic rejection treated, usually unsuccess- some success, but these therapies are generally reserved
fully, with steroids and immunosuppressive agents.34 for patients refractory to corticosteroid therapy. Definitive
Because of the high success rate in treating cases of BOOP therapy is offered by double-lung transplantation, although
unrelated to lung transplant, and because dramatic improve- there is a case report of recurrence of IPH 40 months after
ment is typically seen after a few weeks of therapy with pred- transplantation.35
nisone, patients are unlikely to present for surgery with Evaluation of ongoing alveolar hemorrhage and quanti-
respiratory compromise. These factors also suggest that it fication of the extent of any fibrotic changes is essential for
may be prudent to defer all but the most emergent surgery a complete preoperative assessment. The presence of dys-
in patients just beginning treatment for BOOP. A review of pnea or hemoptysis provides a starting point. Gas exchange
recent radiographs and spirometry, along with a history and is impaired by ongoing alveolar hemorrhage, and there is an
physical examination, typically provide enough information increased need for transfusion in the perioperative period
as to whether the patient's pulmonary function has been opti- because of the acute and chronic loss of red blood cells. It is
mized for elective procedures. prudent to postpone elective surgery until active alveolar
In the event surgery is emergent and cannot be postponed, hemorrhage resolves. Evaluating recent chest radiographs for
the primary anesthetic issues relate to ventilator management. bilateral alveolar infiltrates or new or changing infiltrates will
As in other restrictive lung diseases, high peak pressures may help identify ongoing alveolar hemorrhage. These infiltrates
occur with PPV unless appropriate reductions in tidal volume usually resolve 1 to 2 weeks after the bleeding has stopped.
are made. Rapid arterial hypoxemia can occur with apnea “Honeycombing” may be observed if pulmonary fibrosis has
BOX 4-10 n ANESTHESIA CONCERNS FOR PATIENTS WITH INFILTRATIVE AND INTERSTITIAL DISEASE
Bronchiolitis Obliterans Organizing Pneumonia Systemic Lupus Erythematosus

Assess pulmonary function. Assess all organ system involvement (chest radiograph, PFTs, ABGs,
Tailor anesthetic plan for each patient. BUN/creatinine, LFTs).
Invasive monitors may be indicated if cardiac or pulmonary involvement
Idiopathic Pulmonary Hemosiderosis
and for type of surgery (arterial catheters).
Assess pulmonary function.
Minimize airway manipulation secondary to risk of inflammation and
Evaluate for coagulopathy.
potential laryngeal involvement.
Plan for possible bronchoscopy and pulmonary toilet (use large ETT
Refrain from nitrous oxide use secondary to bone marrow suppression.
when possible).
Ensure thorough evaluation of medications:
May require stress-dose steroids.
Echocardiography may be indicated for cardiac function with high-
Avoid high airway pressures and tidal volumes.
dose cyclophosphamide or hydroxychloroquine.
Chronic Eosinophilic Pneumonia LFTs should be evaluated for hepatotoxicity (azathioprine, methotrexate).
Assess pulmonary function. May require stress-dose steroids.
Delay surgery until steroid therapy implemented. May require increased doses of neuromuscular blockers if taking
May need intraoperative bronchodilators. azathioprine.
Utilize PEEP cautiously and at low levels, if needed at all; minimize Cyclophosphamide may prolong effects of succinylcholine.
intrathoracic pressures to decrease shunt.
Idiopathic Pulmonary Fibrosis
Goodpasture's Syndrome Assess cardiopulmonary function, (PFTs/spirometry, ECG,
Assess cardiopulmonary and renal function (BUN/creatinine, echocardiography).
urinalysis, ABGs, ECG, echocardiography, spirometry). Evaluate for pulmonary hypertension/cor pulmonale.
Maintain oxygenation but limit supplemental O2 to lowest level Aspiration precautions should be considered secondary to association
consistent with arterial saturation >90%. with gastroesophageal reflux disease.
Consider invasive monitors. Acute Respiratory Distress Syndrome
Arterial catheter used for all but the mildest disease; consider TEE or Assess cardiopulmonary function.
PAC if assessment of volume status or adequacy of cardiac output Lung protective ventilation: low tidal volumes (~6 mL/kg predicted body
unclear. weight); PEEP to maintain arterial saturation >90%; <30 cm H2O
Pulmonary Alveolar Proteinosis plateau pressures.
Assess pulmonary function (level of dyspnea, baseline O2 saturation, Utilize permissive hypercapnia as needed.
time since last BPL, ABGs, chest radiograph). Consider invasive monitors (arterial/central venous catheters, TEE).
Double-lumen ETT required for BPL. Provide supportive care, with carefully guided fluid resuscitation.
Invasive monitoring (PAC, TEE) may facilitate intraoperative management Ensure postoperative ventilatory support.
for higher-risk procedures. Pulmonary Histiocytosis X
Sarcoidosis Assess cardiopulmonary function.
Assess all organ system involvement (PFTs, ECG, echocardiography, Tailor anesthetic management to progression of disease.
BUN/creatinine). Give special attention to possible pathologic fractures.
Evaluate airway to rule out lesions by indirect laryngoscopy or CT. Lymphangioleiomyomatosis
Possible postoperative ventilatory support. Assess cardiopulmonary function.
Consider invasive monitors. May need enteral/parenteral nutrition perioperatively.
May require perioperative stress-dose steroids. Consider postoperative ventilation support.
BPL, bronchopulmonary lavage; BUN, blood urea nitrogen; CT, computed tomography; ETT, Endotracheal tube; LFTs, liver function tests; PAC, pulmonary artery
catheter; PEEP, positive end-expiratory pressure; TEE, transesophageal echocardiography.
developed. Preoperative spirometry is recommended, because increased. Corticosteroids or other therapies for IPH should be
a restrictive pattern develops over the course of the disease. If continued throughout the perioperative period (see Box 4-10).
active bleeding is present, the diffusion capacity will be artifi-
cially elevated because of absorption by intra-alveolar hemo-
Chronic Eosinophilic Pneumonia
globin. Anemia frequently develops from ongoing alveolar
hemorrhage, and measuring the amount of serum hemoglo- Chronic eosinophilic pneumonia (CEP) is a rare disorder
bin is essential. of unknown etiology characterized by subacute respiratory
If intubation of the trachea is part of the anesthetic plan, the symptoms caused by infiltration of the alveoli and intersti-
largest possible ETT should be placed to facilitate bronchos- tium by an eosinophil-rich inflammatory process. For the
copy, if needed, and adequate pulmonary toilet. As with other diagnosis to be made, the pneumonia must have no identi-
restrictive processes, higher airway pressures will occur unless fiable cause (e.g., infection, sarcoidosis). CEP is more likely
either a decreased tidal volume is selected or the inspiratory to occur in women and is frequently preceded by adult-onset
phase of ventilation is lengthened. The risk of pneumothorax is asthma. Common presenting symptoms include constitutional
with caution because it may divert blood flow from ventilated

TABLE 4-4 n Sequelae of Idiopathic Pulmonary
Hemosiderosis
alveoli and increase the shunt fraction. Adrenal suppression
may exist because many CEP patients are receiving long-
Sequela Etiology term corticosteroid therapy, and perioperative corticosteroids
should be considered (see Box 4-10).
Recurrent hemoptysis Active alveolar bleeding; very young
children may not be able to
expectorate heme. Goodpasture's Syndrome
Anemia Chronic iron deficiency anemia
Goodpasture's syndrome (GS) is an autoimmune disorder
related to sequestration of
hemosiderin within alveolar that affects the lungs and the kidneys. It is caused by circulat-
macrophages ing anti–glomerular basement membrane (anti-GBM) anti-
bodies that bind to the vascular basement membrane in the
Pulmonary fibrosis Scar tissue and clot formation at
the sites of alveolar hemorrhage
lung and kidneys, resulting in an autoimmune reaction. The
end result is rapidly progressive glomerulonephritis that is
Restrictive lung disease Pulmonary fibrosis frequently accompanied by vasculitis and pulmonary hem-
Pulmonary hypertension Obstruction of pulmonary blood flow orrhage. The incidence is approximately 1 per 100,000 pop-
in interstitial fibrosis ulation, with both genders being affected equally. Genetic
factors are thought to increase the likelihood of developing
Cor pulmonale Pulmonary fibrosis and
hypertension GS, although environmental factors such as smoking, infec-
tion, inhalation injury, volume overload, and exposure to
high oxygen (O2) concentrations increase the risk of pulmo-
c omplaints such as night sweats, weight loss, fever, and cough. nary hemorrhage.38,39 The genetic component of GS is poorly
Progression to dyspnea may occur if not treated. Chest radio- defined. However, there is increased occurrence (88%) of
graphs may show dense peripheral infiltrates, described as a HLA-DR2 in patients with anti-GBM disease compared with
“photographic negative of pulmonary edema.”36 Spirometry in controls (30%). There is also an increased incidence of disease
a symptomatic, untreated patient typically reveals a restrictive in twins, siblings, and cousins of those with GS. Inheritance
pattern. Diffusion capacity is reduced. If bronchospasm is also of certain allelic variants of immunoglobulin heavy chain also
present, the picture may be mixed with a reversible obstruc- increases susceptibility to anti-GBM disease.39 Onset of the
tive component. disease is dramatic, with sudden hemoptysis, dyspnea, and
Corticosteroids are effective treatment for CEP patients, renal failure (Box 4-11). New-onset hypertension may also be
with symptoms often improving in 1 to 3 days and radio- part of the presentation. Renal biopsy is necessary to make
graphic resolution over several months. Unfortunately, recur- the diagnosis and distinguish GS from collagen vascular dis-
rence is common once corticosteroid therapy is discontinued, eases such as WG.
and thus treatment may be needed for life. CEP patients with Because of the sudden onset and severity of the disease, i nitial
concurrent asthma seem to have a lower recurrence rate, pos- treatment frequently requires hemodialysis and mechanical ven-
sibly because inhalation corticosteroids are used as part of tilation. If the GS patient survives the acute phase, high-dose
the management of their asthma.37 The prognosis for CEP is corticosteroids and cyclophosphamide induce immunosuppres-
excellent because of the effectiveness of corticosteroid therapy. sion, and plasmapheresis is used to clear anti-GBM antibodies
If possible, surgery should be delayed until CEP patients have and complement. Therapy usually lasts 3 to 6 months, with reso-
received corticosteroids and experienced resolution of symp- lution of symptoms occurring within the first 2 months. End-
toms, typically 7 to 14 days. stage renal disease is a common complication of GS, and renal
In the event of emergency surgery, the pathophysiologic transplantation may be n ecessary. Early diagnosis and treatment
alterations seen in CEP are similar to those of other pneu- has a strong c orrelation with better outcomes.
monias. Fever may result in reduced intravascular volume
and increased metabolic rate. Fluid resuscitation to restore
euvolemia before induction will decrease the risk of hemody- BOX 4-11 n SYMPTOMS AND SIGNS SEEN IN
namic instability. The increased metabolic rate and increased GOODPASTURE'S SYNDROME
shunt fraction caused by perfusion of inflamed alveoli (which Dyspnea
have impaired gas exchange) will increase the speed of desatu- Fatigue and weakness
ration on induction if apnea develops. Adequate preoxygen- Hematuria
ation and expeditious securing of the airway are therefore Oliguria
Hemoptysis
essential. Intraoperative ventilator management must be
Anemia
individualized, attempting to minimize airway pressures while Hypertension
delivering adequate volumes. If an obstructive component is Azotemia
present, bronchodilator therapy may be helpful, and expira- Proteinuria
tory times may need to be prolonged. PEEP should be used
If possible, surgery should be delayed until medical manage- clubbing, cyanosis, and rales. Definitive diagnosis of PAP
ment is underway and pulmonary involvement has resolved. requires transbronchial or open-lung biopsy. The clini-
In all likelihood, some renal insufficiency, if not failure, will cal course of PAP is variable. Some patients have spontane-
still be present. Preoperative evaluation should include BUN/ ous improvement or remission; others experience persistent
creatinine determinations and urinalysis to assess renal func- but stable symptoms. The other possible clinical course is
tion. The patient's symptoms and medical condition at sur- steady progression of the disease with worsening hypoxia and
gery will dictate the extent of pulmonary evaluation. This may increased risk of infection.
include a chest radiograph, ABG analysis, spirometry, and dif- Chest radiographs typically have bilateral perihilar infil-
fusing capacity to quantify the extent and significance of pul- trates extending into the periphery in a “butterfly” or “bat
monary hemorrhage.40 If pulmonary involvement is ongoing, wing” distribution suggestive of pulmonary edema.43 The
hypoxemia and a restrictive defect on spirometry are com- appearance of the chest radiograph may be out of proportion
mon. A chest radiograph in a typical patient shows diffuse to the severity of the patient's symptoms. High-resolution CT
bilateral alveolar infiltrates from the pulmonary hemorrhage. findings tend to correlate more closely with the clinical pic-
Microcytic anemia from ongoing hemorrhage is also typical. ture. Spirometry frequently reveals a mild restrictive pattern.
Oxygenation is the primary challenge of the anesthetic A severe reduction in diffusing capacity is also observed. ABG
management of patients who have active GS. With ongoing analysis demonstrates hypoxemia and an increased alveolar-
alveolar hemorrhage, patients not only will have impaired gas arterial gradient from interpulmonary shunting.44
exchange at the alveolar level, but also will most likely be ane- Therapy for congenital PAP is supportive; lung trans-
mic. These will contribute to decreased O2 delivery to the tis- plantation is the only definitive therapy currently available.
sues. Exposure of the lungs to an increased O2 tension and Secondary PAP will typically resolve with treatment of the
high airway pressures may exacerbate alveolar hemorrhage. underlying disorder. Whole-lung lavage, also known as bron-
These stresses, along with overaggressive fluid resuscitation, chopulmonary lavage (BPL), has been used in the treatment of
should be avoided in all patients with GS to minimize the risk acquired PAP for 40 years and is still the current standard of
of further anti-GBM–mediated lung injury. An intra-arterial care. More recent reports detail lobar lavage through fiberop-
catheter is indicated when caring for patients with more than tic bronchoscopes in PAP treatment.44 This latter approach is
mild disease. For major procedures in patients with signifi- time-consuming and uncomfortable for the patient and may
cant pulmonary impairment, placement of a PAC or TEE may be most useful in patients who cannot tolerate whole-lung
be helpful in guiding resuscitation and hemodynamic man- lavage or the required general anesthetic.
agement. When selecting anesthetic agents and other medica- A patient with moderate to severe disability caused by
tions, renal function must be considered, and any potentially PAP should be evaluated for the need to have BPL before any
nephrotoxic drugs should be avoided. Dosing of medications elective surgical procedure. Caring for patients receiving BPL
that rely on renal excretion should be altered based on the is significantly easier if the contralateral lung has been recently
patient's creatinine clearance (see Box 4-10). lavaged, because this will dramatically improve oxygenation
during one-lung ventilation, which is required to perform
the procedure. Preoperative testing should be directed by
Pulmonary Alveolar Proteinosis
the patient's level of dyspnea, baseline O2 saturation, and
Pulmonary alveolar proteinosis (PAP) is a rare disorder char- time since the last BPL was performed. In patients with more
acterized by accumulation of a lipoprotein-rich substance in severe symptoms, preoperative ABG analysis or measurement
the alveoli. There appear to be three distinct forms of PAP. of room-air, resting, arterial saturation analysis is indicated.
Congenital PAP presents in infancy and is caused by muta- Chest radiography is unlikely to be useful in evaluating the
tions in the genes coding for surfactant proteins; a defect in extent of disease (see Box 4-10).
surfactant-associated protein B (SP-B) results in accumula-
tion of surfactant-like material in alveoli.41 The secondary BRONCHOPULMONARY LAVAGE
form of PAP involves decreased alveolar macrophage activ- A general anesthetic and placement of a double-lumen ETT
ity, either functional impairment or decreased number, which are required for BPL. A rapid decrease in O2 saturation on
results in decreased clearance of surfactant products and may induction is common, making excellent preoxygenation and
be related to immunosuppression, myeloid disorders, hemato- expeditious placement of the double-lumen ETT essential.
logic malignancies, infection, and inhalation of noxious fumes An intra-arterial catheter is useful in monitoring the patient's
or toxic mineral dusts. Idiopathic PAP does not fit into either oxygenation and hemodynamic response to the procedure.
of the two previous categories and accounts for 90% of cases.42 Confirmation of correct positioning of the ETT by fiberoptic
Idiopathic PAP may also be caused by reduced clearance of visualization is essential. Testing for leaks that would allow
surfactant. The proteinaceous material found in the lungs of contamination of the ventilated lung by spillage of lavage
patients with PAP is surfactant. fluid is critical. The nonventilated lung is then lavaged repeat-
Patients typically present with gradual onset of cough edly with saline while the ipsilateral chest wall is mechani-
and worsening dyspnea with exertion. Chest pain, fever, cally percussed. The procedure is repeated until the drained
and hemoptysis may also be present. Patients may also have saline is almost clear, indicating removal of the majority of
the lipoproteinaceous material. The BPL fluid should be first-degree AV block), ventricular arrhythmias, CHF, peri-
warmed to decrease the risk of hypothermia and the volume carditis, supraventricular tachycardia, ventricular aneurysms,
of the drainage and presence of bubbles closely monitored, to and sudden death.46
ensure isolation of the contralateral lung. Oxygenation may Neurologic findings in sarcoid patients are uncommon,
improve during the instillation of fluid as alveolar pressure although all the nervous system is at risk. Possible manifesta-
increases. This results in decreased perfusion to the lavaged tions of neurologic involvement include seizures, progressive
lung (which is not being ventilated) and thus improves over- dementia, diabetes insipidus, hydrocephalus, and acute mono-
all ventilation/perfusion matching. Hypoxia is most likely to neuropathy. Facial nerve neuropathy is the most common of
occur during the drainage phases of the procedure, when an the neurologic lesions and usually has a benign course.47
increase in intrapulmonary shunting occurs because of the The airways are involved in approximately 5% of patients
dramatic drop in alveolar pressure. Significant hemodynamic with sarcoidosis.48 Symptoms may include dyspnea, dyspha-
changes can also occur during the infusion of saline into the gia, throat pain, hoarseness, a weak voice, or stridor. Most
lung. Hypotension and an increase in central venous pressure lesions are supraglottic and involve the epiglottis, aryepi-
or pulmonary capillary wedge pressure may be seen. TEE glottic folds, and arytenoids.49 These lesions may result in
suggests these changes are caused by impaired venous return airway compromise and, rarely, the need for tracheostomy.
to the left side of the heart.45 Presumably, saline infusion Vocal cord paralysis has also been reported, from recurrent
compresses alveolar capillaries, increasing PVR and result- laryngeal neuropathy caused by sarcoid mediastinal lymph-
ing in increased central venous pressure, while also causing adenopathy.50 Encountering a pregnant patient with a his-
decreased left-sided heart output because of decreased blood tory of sarcoid is not unusual, because sarcoid occurs with an
flow to the left ventricle. In some patients the contralateral increased frequency in women of childbearing age. In general,
lung can be lavaged during the same anesthesia, although however, pregnancy tends to improve sarcoid-related symp-
several days may pass between treatments. BPL may result toms, presumably because of increased cortisol levels during
in improvement lasting 12 to 18 months before it is again pregnancy.51
required. Corticosteroids are often required to treat sarcoid and,
regardless of the lack of correlative data, remain the stan-
dard of care. Systemic corticosteroids appear to improve or
Sarcoidosis
shorten the length of most symptoms related to sarcoidosis.
Sarcoidosis is a chronic granulomatous disease of unknown The relapsing, remitting nature of the disease makes it dif-
etiology that can involve almost any organ system. The diag- ficult to verify the efficacy of this treatment. Data are lim-
nosis is usually made in the first half of adult life, with an ited correlating oral steroids and improved lung function.52,53
occurrence in the United States of 20 to 50 per 100,000 popula Remission occurs within 3 years from diagnosis for more than
tion, with a higher incidence in African-Americans, p eople half of patients with sarcoidosis.54 As is often the case, early
of Northern European descent, and females. The annual diagnosis and treatment appear to improve the likelihood of
mortality rate of a patient with sarcoid is low but is increased successful treatment. Radiation therapy and immunosuppres-
by symptomatic cardiac46 or neurologic47 involvement. The sants such as cyclophosphamide and azathioprine may also be
initial presentation of sarcoid will vary depending on the used. Anti-inflammatory agents, such as anti–tumor necrosis
organ systems affected. Sarcoidosis commonly involves the factor (anti-TNF) therapy (adalimumab) currently are in clin-
skin, eyes, lungs, heart, and CNS. Frequently, abnormal chest ical trials.54
radiographs in asymptomatic individuals raise suspicion. The Serial chest radiographs, PFTs, and serum angiotensin-
lesions responsible for sarcoidosis are noncaseating granulo- converting enzyme (ACE) levels can be used to follow the
mas, which may spontaneously resolve or proceed to fibrosis. progress of a patient. Serum ACE appears to be synthesized
The vast majority of sarcoid patients have pulmonary within sarcoid granulomas. High levels are associated with
involvement. Many are asymptomatic, whereas others will more severe pulmonary infiltration, and lower levels are seen
have nonspecific complaints such as chest pain, dyspnea, and with disease inactivity. Trends within a given patient are more
nonproductive cough. Radiographic abnormalities progress important than the absolute level of ACE. Cardiac rhythm
from bilateral hilar adenopathy to diffuse pulmonary infil- abnormalities can result from sarcoid heart disease and may
tration, and in severe cases, pulmonary fibrosis. PFTs fre- necessitate placement of a pacemaker or implantable cardiac
quently demonstrate restrictive disease with decreased lung defibrillator, as well as other treatment for arrhythmias, car-
volumes and diffusion capacity. In some cases an obstructive diomyopathy, and heart failure.
pattern may also be present because of airway narrowing. In Preparation for anesthesia in a patient with a history of
more advanced cases, ABG analysis reveals hypoxemia and sarcoidosis should focus on the airway and pulmonary func-
an increased alveolar-arterial gradient. A significant num- tion, as well as on evaluation of other organ systems known to
ber of sarcoid patients have cardiac symptoms resulting from have been affected in the individual patient. A review of recent
myocardial granulomas or the effects of respiratory system chest radiographs along with PFTs is recommended. A history
disease on the heart. Possible findings include conduction of significant dyspnea warrants an ABG analysis. Screening
abnormalities (complete heart block, bundle branch block, or for airway involvement can be accomplished by inquiring
about dysphagia, hoarseness, or throat pain. If suspected, an Pericarditis, small pericardial effusions, valvular abnormalities,
evaluation by indirect or direct laryngoscopy and, if necessary, and endocarditis represent the majority of the cardiac mani-
head and neck CT will provide the necessary anatomic data. festations. CHF may occur, although usually not the result of
Swelling of supraglottic structures may increase the difficulty cardiomyopathy.55
of intubation and increase the risk of postoperative respiratory Treatment is typically directed at specific symptoms,
compromise. Delaying surgery to allow for adequate cortico- including nonsteroidal anti-inflammatory drugs (NSAIDs)
steroid therapy may be appropriate. Other preoperative test- for arthritic pain, glucocorticoids for anemia and thrombo-
ing is guided by the patient's history and may include ECG cytopenia, anticonvulsants for seizures, anticoagulants for
and echocardiogram if cardiac involvement or advanced pul- thrombosis, and dialysis for end-stage renal disease. Other
monary fibrosis is present. All ongoing cardiac therapy should treatments may include plasmapheresis, azathioprine, and
be continued perioperatively. Because of the sporadic nature cyclophosphamide.
of neurologic symptoms, a thorough neurologic examination Pulmonary manifestations of SLE are the direct result of
is advisable during preoperative evaluation to help differenti- autoantibody reactions in the lung vasculature, lung paren-
ate between existing deficits and those resulting from anes- chyma, and pleura (Table 4-5).56–59 Laryngeal complications
thetic interventions, surgery, or positioning for surgery. Renal in SLE have an incidence of 0.3% to 30% and range from
involvement also occurs, making review of recent electrolyte mild inflammation to vocal cord paralysis, subglottic ste-
and renal function data advisable. nosis, and acute obstruction from edema.56 Therefore, thor-
Intraoperative management of an asymptomatic patient ough airway evaluation and history is crucial preoperatively.
should be uneventful and require little change in the anesthetic Histopathologic findings include alveolar wall damage, inflam-
plan when compared with a healthy individual undergoing the matory cell infiltration, hemorrhage, and hyaline membranes.
same procedure. The patient with significant restrictive lung Some manifestations are thought to occur primarily in SLE
disease will require altered ventilator management and pos- patients with antiphospholipid antibodies, the presence of
sible postoperative ventilatory support. An intra-arterial cath- which is known as antiphospholipid syndrome (APS); 50% of
eter facilitates oxygenation and ventilation management and APS cases occur in patients with SLE, although only a minority
allows close observation and early detection of any hemody- of SLE patients have APS.57 The primary defect in APS is recur-
namic instability. In caring for patients with significant pul- rent arterial and venous thrombosis.58 However, APS is also
monary fibrosis, placement of a PAC or use of TEE may help associated with pulmonary hypertension and diffuse alveolar
guide resuscitation and hemodynamic management. Sarcoid
patients with an implantable cardiac defibrillator may need
to have these devices inactivated because of interference from TABLE 4-5 n Systemic Lupus Erythematosus (SLE):
electrocautery units, although modern units are less suscep- Pulmonary Manifestations
tible. In patients with deactivated devices, defibrillator pads Finding Comment
should be placed during the period of inactivation to allow for
external pacing and defibrillation, if needed. Airway manage- PRIMARY MANIFESTATIONS
ment will be dictated by the preoperative evaluation; awake Lupus pneumonitis Mimics acute infectious pneumonia
fiberoptic intubation or elective tracheostomy is occasionally
Diffuse alveolar Rare; may be associated with APS
necessary. Continuation of corticosteroid therapy with con-
hemorrhage
sideration of stress dosing is encouraged.
Lupus pleuritis Pleurisy and pleural effusion are
common in SLE
Systemic Lupus Erythematosus
Interstitial pneumonia Includes lymphocytic and BOOP
Systemic lupus erythematosus (SLE) is a connective tissue dis- variants
ease resulting from autoantibodies directed at cellular nuclei Pulmonary hypertension Resembles PPH; associated with APS
antigens found in multiple organ systems. The cause of SLE
is unknown. SLE can occur in anyone but most often affects Bronchiolitis Rare and unexplained
women of childbearing age. Its incidence is estimated at 40 per Chronic interstitial lung Resembles idiopathic pulmonary
100,000 population in North America. disease fibrosis
Arthritis is the most common clinical manifestation of SLE.
SECONDARY MANIFESTATIONS
Other common signs and symptoms include cutaneous lesions
such as butterfly malar erythema, Raynaud's phenomenon, Pulmonary embolism Caused by recurrent thrombosis
oral ulcers, and recurrent noninfectious pharyngitis. Anemia, associated with APS
thrombocytopenia, leukopenia, and an increased incidence Respiratory muscle Subsegmental atelectasis; elevated
of thrombus formation are possible hematologic sequelae. dysfunction diaphragm; “shrinking lung”
Renal involvement, in the form of glomerulonephritis, has a syndrome
highly variable course. Neurologic findings with SLE include APS, Antiphospholipid syndrome; BOOP, bronchiolitis obliterans organizing
cognitive dysfunction, migraine-like headaches, and seizures. pneumonia; PPH, primary pulmonary hypertension.
hemorrhage, particularly dire manifestations that predict a

TABLE 4-6 n Idiopathic Pulmonary Fibrosis:
higher mortality.59 The majority of these patients respond to Experimental Therapies
immunosuppressive therapy and rarely require emergency air-
way intervention.56 Therapy Action
Preoperative testing should be directed toward the
Interferon-γ 1b Inhibition of fibroblast proliferation and
affected organ systems. Many SLE patients have mild dis- collagen synthesis
ease and require little deviation from the routine periop-
erative evaluation and care required for a given procedure. Pirfenidone Inhibits synthesis of collagen and tumor
necrosis factor alpha
A review of serum BUN/creatinine levels is reasonable to
rule out any occult renal involvement. Pulmonary evalu- Acetylcysteine Stimulates glutathione synthesis
ation may include chest radiography, ABG analysis, and
Data from Selman M, Thannickal VJ, Pardo DA, et al: Idiopathic pulmonary
PFTs if current symptoms and history suggest pleuropulmo- fibrosis: pathogenesis and therapeutic approaches, Drugs 64:405-430, 2004.
nary involvement. A restrictive pattern is frequently seen on
PFTs, although patients with bronchiolitis will have obstruc-
tion as well. The diffusing capacity is reduced when inter- transplantation.64 The median survival time is 2 to 3 years
stitial disease is present. Diffusing capacity is normal when from time of diagnosis.65
corrected for diminished lung volumes if respiratory muscle Patients with IPF presenting for surgery typically are
dysfunction is the sole cause of underlying restrictive lung tachypneic and cyanotic and appear to be in poor health.
disease.60 Patients with significant pulmonary involvement Preoperative evaluation should include a review of recent spi-
may require postoperative ventilation. Ventilator manage- rometry and other PFTs. A decrease in lung volumes with a
ment should be tailored to their specific disease process: reduction in diffusion capacity is expected. Ventilation/per-
diaphragmatic weakness or interstitial fibrosis. During the fusion inequality and impaired diffusion result in hypox-
perioperative period, patients with APS are at increased risk emia. In patients with advanced disease, echocardiography
of thrombosis, and appropriate precautions must be taken. may reveal pulmonary hypertension and cor pulmonale. IPF
Perioperative corticosteroids may be required for patients patients seem to have a very high incidence of GERD.66 It is
with adrenal insufficiency because of chronic corticosteroid appropriate to consider premedication to reduce gastric vol-
administration (see Box 4-10). ume and acidity, as well as an anesthetic technique to min-
imize the risk of pulmonary aspiration of gastric contents.
An aspiration event in such a patient could easily be fatal.
Idiopathic Pulmonary Fibrosis
Placement of an intra-arterial catheter is advised for all but
Idiopathic pulmonary fibrosis (IPF), also referred to as “cryp- the most vigorous of these patients undergoing minor surgery
togenic fibrosing alveolitis,” is an interstitial lung disease of (see Box 4-10).
uncertain etiology. IPF is a progressive illness with a median Patients with IPF are most likely to present to the operating
survival of 3 to 4 years. This rare condition has a prevalence room (OR) for lung biopsy to establish the diagnosis, for lung
of about 5 per 100,000 population and is more common in transplant in a curative effort, or for resection of a pulmonary
current or former smokers. A typical patient is a middle-aged neoplasm. These procedures usually require one-lung ventila-
man. Diagnosis is based on the histologic pattern of usual tion, a challenge in patients with advanced disease. Placement
interstitial pneumonia and exclusion of other causes of this of a double-lumen ETT will provide the added ability to pro-
histologic pattern. Extrapulmonary involvement does not vide passive oxygenation to the nonventilated lung in an effort
occur. The presentation is insidious and typically involves to minimize hypoxemia. Patients with advanced disease may
dyspnea and a nonproductive cough. Physical examination require postoperative care in an intensive care unit (ICU) and
frequently reveals fine crackles at the lung bases, expanding possible mechanical ventilation.
upward as the disease progresses. Clubbing, cyanosis, periph-
eral edema, and cor pulmonale are later findings. There must
Acute Respiratory Distress Syndrome
be a restrictive pattern on spirometry and radiologic changes
on chest radiography or high-resolution CT consistent with Acute respiratory distress syndrome (ARDS) is a severe form
the diagnosis.61 of acute lung injury resulting from an underlying illness
Patients with IPF also have an increased incidence of pul- or lung injury. ARDS may occur in as many as 10 to 20 per
monary malignancy. Unfortunately, it is unclear if resection 100,000 individuals.67 Several disorders are implicated as risk
of these lesions adds to life expectancy in this population.62 factors for developing ARDS, through direct lung injury or
No effective treatment is currently available, although cortico- a systemic inflammatory response (Table 4-7). The underly-
steroid and cytotoxic agents are frequently used. Many novel ing lesion is injury to the alveolar-capillary membrane and
therapies attempt to block fibrogenic pathways and may be of increased membrane permeability. Proteinaceous edema
benefit (Table 4-6).63 Lung transplantation is the only thera- fluid accumulates in the alveoli, resulting in impaired oxy-
peutic option available for IPF patients. Although thought to genation and poorly compliant (stiff) lungs. ARDS develops
be effective, there is still only a 49% survival rate 5 years after acutely over 1 to 2 days. If a patient is alert and spontaneously
be inserted. For procedures involving major fluid shifts,

TABLE 4-7 n Acute Respiratory Distress Syndrome:
Associated Clinical Disorders
placement of a PAC or use of TEE may be helpful in guid-
ing resuscitation and avoiding overzealous fluid administra-
Direct Lung Injury Indirect Lung Injury tion, which might adversely impact the patient's respiratory
status. However, ARDS patients in an ICU do not have bet-
Aspiration of gastric contents Sepsis
ter outcomes when managed with a PAC as opposed to a cen-
Inhalation of toxic fumes Major trauma tral venous catheter.73 Neutral fluid balance can also benefit
Near-drowning Reperfusion injury
patients with ARDS, although appropriate fluid resuscitation
and maintenance of vital organ perfusion usually preclude
Pulmonary contusions Massive transfusions such an intraoperative fluid management strategy. Colloids
Diffuse pulmonary infection Drug overdose such as albumin and hetastarch offer no advantage over crys-
talloid solutions because impaired alveolar-capillary mem-
Data from Hudson LD, Steinberg KP: Acute respiratory distress syndrome:
clinical features, management and outcome. In Fishman AP, et al, editors:
branes allow both classes of fluid to reach the extravascular
Fishman's pulmonary diseases and disorders, New York, 1998, McGraw-Hill, space (see Box 4-10).
p 2550.
Pulmonary Histiocytosis X
ventilating, anxiety and dyspnea will be the earliest signs. As
inflammatory changes occur, tachypnea and increased work of Pulmonary histiocytosis X (PHX), also called “pulmonary
breathing will be noted. Langerhans cell granulomatosis,” is an uncommon interstitial
Mechanical ventilation is required to maintain oxygen- lung disease associated with cigarette smoking. Related disor-
ation. Chest radiographs typically reveal diffuse bilateral alve- ders are Hand-Schüller-Christian and Letterer-Siwe diseases.
olar infiltrates similar to the findings of pulmonary edema. The primary defect appears to be the pathologic accumulation
There is no laboratory test to diagnose ARDS. As a clinical of Langerhans cells around bronchioles and the pulmonary
diagnosis, criteria for diagnosing ARDS are acute onset of vasculature, leading to the formation of granulomas and fibro-
respiratory distress requiring intubation and mechanical ven- sis. Most PHX patients present in early adulthood and have
tilation; a Pao2/Fio2 ratio of less than 200, a chest radiograph a history of cigarette smoking, with men and women equally
with bilateral infiltrates suggestive of pulmonary edema, and affected.
no evidence of CHF or, if measured, a pulmonary artery Presenting symptoms are nonspecific and include nonpro-
wedge pressure less than 18 mm Hg.68 Although it has declined ductive cough, dyspnea, fatigue, fever, and weight loss (Table 4-8).
over the past 10 years, ARDS still has a high mortality rate of Reticulonodular infiltrates, upper-lobe and middle-lobe cysts,
25% to 35%. Patients who do survive generally return to a pul- and stellate nodules with sparing of the costophrenic angle on
monary function near their baseline. Any remaining defect is chest radiography highly suggest PHX; bronchoalveolar lavage
likely restrictive or involving decreased diffusion capacity, and (BAL) or biopsy confirms the diagnosis. Results of spirometry
more disabling sequelae are possible.69 may yield an obstructive, restrictive, mixed, or normal pattern.
Intraoperative management of ARDS is an extension of the A decrease in diffusion capacity appears to be the most consis-
patient's ICU care. Many patients have a severe underlying tent finding. Physical limitation in PHX patients is frequently out
injury or illness, which also requires significant perioperative of proportion to spirometry results, and pulmonary hypertension
attention. The approach to ventilator management plays a sig-
nificant role in ARDS mortality. Instituting low–tidal volume
ventilation of 6 mL/kg (predicted body weight) and main- TABLE 4-8 n Pulmonary Histiocytosis X: Associated or
taining plateau pressures of less than 30 cm H2O were found Causal Comorbidities
to reduce mortality by almost 20%.70,71 This approach may
Condition Issues
result in hypercapnia and respiratory acidosis, which can be
monitored and treated with sodium bicarbonate. Permissive Spontaneous pneumothorax May be recurrent
hypercapnia is usually well tolerated and may reduce mortal-
Hemoptysis secondary to Rare
ity from lung injury.72 There is no clear evidence to support aspergillosis
that pressure-cycled ventilation is superior to volume-cycled
ventilation. Primary lung tumors Causative relationship is
unclear.
Administration of PEEP is necessary and results in recruit-
ment of alveoli and better ventilation/perfusion matching. Secondary pulmonary Common, may result in cor
No set level of PEEP has been shown to be superior.71 Other hypertension pulmonale
maneuvers, such as sigh breathing and periodic rotation of Central diabetes insipidus Occurs with central nervous
the patient to the prone position, may result in improved oxy- system involvement
genation but are not associated with improved outcomes.
Cystic bone lesions Cause bone pain and
Invasive monitors will frequently be in place when the patient pathologic fractures
arrives in the OR; if not, an intra-arterial catheter should
may play a significant role in contributing to diminished exercise capacity. Exercise capacity will be severely decreased because of
capacity. In advanced disease, pulmonary artery pressures in the ventilation/perfusion inequality and increased work of breath-
range of 60 mm Hg are not unusual.74 ing.77 ABG analysis typically reveals a decrease in Po2 and Pco2,
The course of PHX is unpredictable. Improvement or com- although pH is normal.78
plete remission may occur spontaneously or as the result of Estrogen is thought to play a role in the development of
smoking cessation. A minority of patients progress to pulmo- LAM because of its almost exclusive occurrence in women of
nary fibrosis. Age at presentation (>26 years), forced expiratory childbearing age, its exacerbation by pregnancy, and pres-
volume in 1 second/forced viral capacity (FEV1/FVC) ratio less ence of estrogen receptors on biopsy tissue. Recently, rapamy-
than 0.66, and right ventricular/total lung capacity (RV/TLC) cin (sirolimus) has been suggested as a therapeutic option
ratio greater than 0.33 are cited as predictors of advanced dis- for LAM. The results are not consistent, and some studies
ease and increased mortality.75 Corticosteroids and chemo- show significant reduction in size of angiomyolipomas and
therapeutic agents are used in attempts to treat PHX, but the improvement of lung function.79 Corticosteroids are ineffec-
disease is frequently refractory to treatment. Lung transplan- tive. Modalities to block the molecular effects of estrogen (e.g.,
tation has been performed with success, although PHX has doxycycline) have been somewhat more successful. These
recurred in the transplanted lungs of patients who had extra- approaches include oophorectomy, progesterone, and tamoxi-
pulmonary involvement and had resumed smoking.76 fen. Lung transplantation is offered to patients with advanced
Patients who are in remission or have only mild symptoms disease, although this is frequently complicated by disease-
do not require special preoperative evaluation or intraoper- associated problems such as pleural adhesions, postopera-
ative management beyond that warranted by the scheduled tive chylothorax, pneumothorax, and recurrent LAM.79 Lung
procedure. In patients with more advanced disease, a review transplant may be considered as an option for end-stage pul-
of PFT results and ABG analysis and evaluation of pulmonary monary LAM. Survival after lung transplant is 79% at 1 year
pressures by echocardiography or direct measurement are and 73% at 3 years.80
recommended. Based on these results, intraoperative man- Preoperative evaluation should include a review of
agement should be tailored to avoid increases in pulmonary recent PFTs and chest radiographs, as well as ABG analy-
artery pressure. Placement of a PAC may be necessary to help sis in advanced cases. Elective surgery should be postponed
achieve this goal. The risk of pneumothorax in this population until after significant chylothorax, if present, can be drained
warrants an effort to minimize peak airway pressures. If dia- and chest tubes inserted to resolve existing pneumothoraces.
betes insipidus is present, treatment with desmopressin should Recurrent leakage of lymph results in an impaired immune
be continued perioperatively. The potential for pathologic response and nutritional wasting, which increase the patient's
fractures from cystic bone lesions requires special attention to risk of perioperative complications and should be addressed
patient positioning and padding. As in all patients with pul- before surgery by enteral or parenteral nutritional support.
monary disability preoperatively, the potential for postopera- For patients with advanced disease, ventilator management
tive ventilatory support should be factored into the anesthetic should be similar to that for a patient with severe emphysema,
plan and discussed in advance with the patient (see Box 4-10). including prolonged expiratory time and avoidance of high
inspiratory pressure. Postoperative ventilatory support may
be required if the patient has severe underlying disease and is
Lymphangioleiomyomatosis
undergoing major or extensive surgery. Placement of an intra-
Lymphangioleiomyomatosis (LAM) is a rare, progressive inter- arterial catheter is helpful in obtaining serial ABGs to guide
stitial lung disease of unknown origin that frequently leads to ventilator management (see Box 4-10).
deteriorating lung function and death secondary to respiratory
failure. LAM occurs in women of reproductive age and is exac-
erbated by pregnancy. It also occurs in male and female patients ARTHRITIC DISEASES CREATING UPPER
with tuberous sclerosis. The condition results from the prolif- AIRWAY AND RESPIRATORY PROBLEMS
eration of interstitial smooth muscle and formation of cysts,
which obliterate and obstruct the airways. Complaints of dys-
pnea are the typical presenting symptom. Individuals with LAM Ankylosing spondylitis (AkS) is a chronic inflammatory pro-
develop hyperinflated lungs with an increased total lung capac- cess of unknown etiology that primarily deforms the axial
ity. They also develop an obstructive pattern on spirometry. skeleton, resulting in fusion. The disease is predominantly
Spontaneous pneumothorax caused by cyst rupture is common. diagnosed in young adults, with men more likely to be affected
Obstruction and eventual rupture of the thoracic duct, resulting than women. Prevalence in the United States is about 1 in 1000
in chylothorax, is another manifestation of LAM. Hemoptysis individuals. AkS apparently has a genetic component, because
occurs infrequently. Chest radiographs are normal appearing most affected individuals are HLA-B27 positive.
early in the disease but resemble those of end-stage emphy- As a result of chronic inflammatory changes at the ligamen-
sema in advanced disease. Reticulonodular opacities may also tous insertions onto bone, the vertebrae begin to grow into
be seen. An obstructive or occasionally a mixed pattern is pres- each other, forming outgrowths known as syndesmophytes.
ent on spirometry, along with a significant decrease in diffusion These changes result in the appearance of a “bamboo spine”
in radiologic evaluation and decreased mobility of the spine. assess the extent of fusion. Caution should be exercised when
This process generally begins in the sacral and lumbar regions, instrumenting the airway because of involvement of the cervical
with cervical involvement occurring much later in the disease spine. Decreased range of motion and poor mouth opening can
course. Extraskeletal manifestations of AkS may occur, par- make direct laryngoscopy difficult, and excess force applied to
ticularly peripheral joint manifestations; although generally the neck can result in cervical fracture. Atlantoaxial subluxation
uncommon, these include aortic insufficiency, cardiac con- is also present in a subset of these patients.86 If advanced disease
duction abnormalities, iritis, upper-lobe fibrobullous disease, is present, an alternative and conservative approach to airway
and pleural effusions. Risk of aspergilloma and hemoptysis is management (including an awake intubation) is strongly recom-
high if fibrobullous disease develops.81 mended, preferably one that maintains spontaneous ventilation.
Involvement of the sternocostal, costovertebral, and tho- Adjuncts such as a laryngeal mask airway (LMA), videolaryn-
racic spine results in decreased mobility of the thoracic cage goscope, and fiberoptic bronchoscope should be readily avail-
and a restrictive ventilatory pattern. Although common in able. Neuraxial anesthesia is very challenging in AkS patients.
AkS patients, decreased exercise tolerance is thought to be The ossification of spinal ligaments significantly narrows or even
caused by deconditioning as opposed to a primary pulmonary closes the intervertebral space and prevents optimal positioning.
defect.82 The limitation in thoracic cage movement is almost Alternatives reported as successful include a lateral approach to
totally compensated for by increased diaphragmatic excur- spinal placement87 and placement of caudal catheters.88
sion.83 As the disease progresses, exercise tolerance also is Intraoperative management must include special attention
decreased because of the restrictive lung process. to positioning because of the inflexibility of the AkS patient's
Historically, treatment for AkS was symptom based and spine. Diaphragmatic function should be optimized dur-
relied on NSAIDs and physical therapy to reduce back pain ing spontaneous ventilation because of a restrictive thoracic
and stiffness. Using NSAIDs for long-term therapy poses an cage. This can be accomplished by avoiding the Trendelenburg
increased risk of peptic ulcers and gastritis in this population. position and using large-diameter ETTs when possible.
For this reason, cyclo-oxygenase (COX-2) inhibitors have been Interscalene blocks can result in short-term ipsilateral dia-
increasingly used as an alternative. The controversy regarding phragmatic paralysis and should be avoided. Higher peak
the cardiovascular safety of COX-2 inhibitors indicates careful pressures may occur with PPV and are expected. Adequate
consideration of the risks and benefits. Sulfasalazine, meth- ventilation during laparoscopic surgery may not be possible,
otrexate, and corticosteroids are used in severe cases. Most and hypercarbia may develop; if not excessive, it can be toler-
recently, more than a third of AkS patients were in remission ated until the end of the procedure. Strictest extubation cri-
after 5 years of continuous TNF inhibitors therapy.84,85 teria should be observed in patients with AkS, because their
heavy reliance on diaphragmatic function increases their risk
ANESTHESIA MANAGEMENT of postoperative respiratory insufficiency, and emergent rein-
A patient with advanced AkS presents a significant challenge tubation carries a significant risk of morbidity and failure.
to the anesthesiologist, frequently needing orthopedic proce-
dures on the hips and knees (Box 4-12). Preoperative e valuation
Kyphosis and Scoliosis
should include radiographs of the lower and cervical spine to
Scoliosis is a lateral and rotational deformity of the spine that also
results in deformity of the rib cage. Kyphosis is an exaggerated
BOX 4-12 n ANESTHESIA CONCERNS FOR PATIENTS anterior flexion of the spine resulting in a rounded or hump-
WITH ARTHRITIC DISEASE backed appearance. These disorders are frequently seen together
and are referred to as kyphoscoliosis. The vast majority of cases
Assess cardiopulmonary function. can be classified as idiopathic, congenital, or neuromuscular.
Review radiographic imaging to determine the significance of cervical The idiopathic form is the most common and is more likely to
spine disease before airway management and positioning that occur in women than men. Corrective surgery is performed for
necessitate movement of the neck. scoliosis when spinal angulation, also known as the Cobb angle,
Use cautious manipulation of the neck because of instability and
mobility limitations.
exceeds 50% in the thoracic or 40% in the lumbar spine.89
These patients should be regarded as having a “difficult airway.” Preoperative assessment should focus on any cardiovas-
Neuroaxial anesthesia is very challenging. cular, respiratory, or neurologic impairment related to the
Give special attention to positioning. deformity. Restrictive lung disease is common, the result of
Kyphosis and Scoliosis a narrowed chest cavity. Although patient history will pro-
Assess cardiopulmonary and neurologic function. vide significant insight into the level of disability, PFTs and
May have significant blood loss during surgery to correct either ABG analysis are crucial in evaluating the extent of restric-
condition.
tion and hypoxemia. This information will guide deci-
May have one-lung ventilation.
Deliberate hypotension may be requested intraoperatively. sions regarding postoperative ventilatory support. PFTs are
Take special care with positioning. likely to demonstrate a reduced vital capacity and total lung
Consider intraoperative neurophysiologic monitoring (SSEP, MEP). capacity, as well as a normal residual volume. Hypoxemia
results from ventilation/perfusion inequality. Patients may
also h ypoventilate. Cor pulmonale resulting from chronic compromise to the spinal cord. Data obtained by TEE and venous
hypoxemia and pulmonary hypertension may be present in oxygen saturation (SvO2) monitoring suggest that spinal cord
advanced cases. These concerns make electrocardiography, ischemia that results from distraction of the spine is the result of
echocardiography, and, in some situations, an exercise stress both direct compression of the spinal cord as well as decreased
test reasonable components of preoperative testing. A his- cardiac output and decreased BP caused by compression of vena
tory and physical examination is sufficient to evaluate the cava or the heart.92 Intraoperative neurologic monitoring has
patient's neurologic status. It is important to document any become the standard of care for procedures involving signif-
pre-existing neurologic deficits so as to differentiate between icant distraction of the spine. Patient temperature, pH, and
baseline deficits and those resulting from surgery. This is also adequate BP must all be maintained within narrow limits to
helpful in minimizing further injury secondary to position- maximize the effectiveness of SSEP monitoring. Controversy
ing or airway management. surrounds the preferred anesthetic agents with SSEP moni-
Corrective spinal surgery is the procedure most likely to toring. The literature is frequently contradictory, and institu-
bring these patients to the OR. The many variations include tional preferences vary greatly, although propofol infusions
anterior, posterior, and combined approaches, as well as lum- and nitrous oxide are popular. The most important factor does
bar and thoracic level repairs. A combined anterior/posterior appear to be administration of a stable anesthetic, with mini-
approach under a single anesthetic has a higher rate of major mal bolus dosing and close communication with the clinician
complications than a staged procedure and is best avoided if monitoring the evoked potentials (see Box 4-12).
possible.90 Many elements of the anesthetic plan, such as posi-
tioning and the need for one-lung ventilation, will be dictated
by the specific procedure. DRUG-INDUCED LUNG INJURY
Bleomycin Toxicity
ANESTHESIA MANAGEMENT
Despite differences in the types of spinal surgery, several con- Bleomycin is an antineoplastic antibiotic used in combination
cerns apply to all. These procedures frequently involve signifi- chemotherapy for a number of malignancies, including Hodgkin's
cant blood loss, possible one-lung ventilation, and the need lymphoma, Wilms’ tumor, and testicular cancer. Although effec-
for deliberate hypotension. The patients have underlying pul- tive in treating bacterial and fungal infections, bleomycin is not
monary restrictive disease. All of these factors make arterial used for these purposes because of its cytotoxicity. The appeal of
line placement and ABG analysis critical to effective periop- bleomycin in combination chemotherapy protocols is its lack of a
erative management. The presence of restrictive lung disease myelosuppressive effect. This avoids adding to the bone marrow
combined with prone or lateral positioning can make oxygen- toxicity common to other antineoplastic agents.
ation and ventilation with acceptable peak airway pressures Unfortunately, bleomycin carries the risk of inducing pul-
challenging. The use of an anesthesia machine or ventilator monary toxicity, which can result in pulmonary fibrosis and
capable of pressure control ventilation may be helpful. Based can be life threatening. Total dose received relates to the
on the level of preoperative disability, the need for postopera- extent of pulmonary toxicity in animals, but this is less clear
tive ventilation should be discussed with the patient and fam- in humans. There is no consensus on a cumulative dose that
ily. Of note, adequate oxygenation during one-lung ventilation increases risk, although more than 300,000 IU is the suggested
for anterior thoracic approaches may be difficult. Placement threshold.93 Intravascular administration may be a risk fac-
of a double-lumen ETT instead of a bronchial blocker offers tor compared with intramuscular dosing.94 Chest irradiation
the advantage of delivering passive oxygenation to the non- in conjunction with bleomycin therapy appears to increase
ventilated lung. However, such tubes have the disadvantage of the risk of bleomycin-induced pulmonary fibrosis, as does
needing to switch to a single-lumen ETT at the end of the pro- advanced age, a history of smoking, and treatment with other
cedure if postoperative ventilation is required. Improvement chemotherapeutic agents that have pulmonary toxicities, such
in the patient's pulmonary function does not occur immedi- as busulfan, carmustine, semustine, and lomustine. Impaired
ately after surgery, and any improvement may take months to renal function increases the risk of toxicity by reducing the
several years, depending on the procedure.91 elimination of bleomycin from the body.
Large-bore venous access, central or otherwise, is needed to Pulmonary toxicity caused by bleomycin results in a simi-
ensure rapid replacement of intraoperative blood loss. Central lar pulmonary fibrosis as seen in IPF. Patients usually present
venous pressure monitoring is of limited usefulness in these pro- with a nonproductive cough accompanied by dyspnea. Chest
cedures because of the effects of positioning on the values obtained radiographs initially reveal bibasilar infiltrates, but as the pro-
and possible pulmonary hypertension or cor pulmonale, which cess continues, the radiograph will take on a “honeycomb
reduces the value of central venous pressure monitoring in deter- lung” appearance. PFTs will have a restrictive pattern in symp-
mining the adequacy of intravascular volume. TEE is a reasonable tomatic patients but are of little predictive value in asymptom-
choice to monitor intravascular volume status and cardiac con- atic patients who have been exposed to bleomycin.
tractility, if available and if the patient's position allows. Evaluation of the bleomycin patient for anesthesia focuses
Spinal cord monitoring such as somatosensory evoked poten- on pulmonary function, symptoms (e.g., dry cough, dyspnea,
tials (SSEPs) and, to a lesser extent, motor evoked potentials decreased exercise tolerance), and risk factors (e.g., large
(MEPs) are frequently used to detect direct trauma or vascular cumulative dose, chest radiation, smoking). If the patient
denies symptoms, chest radiographs, PFTs, and ABG analysis died during the pandemic in Spain in 1918, and 1 million died
are not likely to be useful. Symptomatic patients require testing in Hong Kong in 1968.96 Both were the result of an influenza
to quantify their disability, plan appropriate perioperative care, A strain variant (H1N1). Influenza viruses undergo continual
and determine the need for postoperative ventilatory support. antigenic drift, aiding in their ability to resist the host's immu-
nity. This contributes to the continued pandemics of viruses,
ANESTHESIA MANAGEMENT particularly influenza A strains. Most recently, the H1N1
A landmark study has guided the anesthetic management of strain led to catastrophic mortality, particularly in the infant
bleomycin patients for 35 years. Goldiner et al.95 implicated and young population. First identified in Mexico in 2009,
hyperoxia and fluid overload as increasing the risk of periop- H1N1 quickly spread worldwide. This variant is composed of
erative pulmonary morbidity and mortality in patients who swine, bird, and human strains of influenza A. The rates of
received bleomycin. Although subsequent studies have ques- transmission are higher than for the seasonal influenza. The
tioned these guidelines, there is no reason to believe that pro- main route of transmission is human-to-human exposure
viding a higher fraction of inspired oxygen (Fio2) than that through large respiratory droplets or contaminated surfaces.
needed to maintain adequate oxygenation is of any benefit Patients present with a combination of flulike symptoms,
to these patients. The one exception to this is during preoxy- including fever, cough, shortness of breath, fatigue, diarrhea, and
genation, which is relatively brief, before induction of general vomiting. Associated comorbidities include asthma, obesity, and
anesthesia.94 When adequate oxygenation does require an Fio2 diabetes mellitus. Infants and children are at increased risk, as
greater than 30%, use of PEEP may facilitate oxygen action are patients with chronic health conditions, those receiving renal
without necessitating higher levels of Fio2. Fluid therapy replacement therapy, and pregnant women. Concomitant com-
should be conservative, with the goal of maintaining adequate plications include myocarditis, encephalitis, ARDS, refractory
intravascular volume and avoiding excess fluid administra- hypoxemia, and secondary bacterial infections (e.g., sepsis).
tion. There is no evidence to support the use of colloid instead A few diagnostic tests are available for influenza A, but
of crystalloid in bleomycin patients. When significant blood treatment should not be delayed awaiting results. Patients with
loss or significant fluid shifts are expected in surgery, intra- a high index of suspicion should be treated without a confir-
arterial and central venous catheters may be helpful. There is matory test. A provisional diagnosis can be established within
no clear answer as to how long after completion of therapy 30 minutes to 1 hour by the rapid influenza diagnostic test
with bleomycin a patient continues to be at risk for pulmonary (RIDT). However, both RIDT and direct immunofluorescent
fibrosis, although minimizing Fio2 for 1 to 2 years would seem assay (DFA) are unable to differentiate between pandemic and
prudent (Box 4-13). seasonal influenza variants and have lower sensitivity than real-
In patients with documented pulmonary bleomycin tox- time reverse-transcriptase polymerase chain reaction (rRT-
icity, higher-than-normal peak pressures are expected with PCR) and viral culture. Confirmation of pandemic virus can
PPV, although this may be necessary for adequate oxygenation be determined only by rRT-PCR or viral culture (Table 4-9).
and ventilation. Strict extubation criteria should be observed Prophylactic treatment is of no benefit and increases the
because these patients are at increased risk of postoperative risk of resistance to antiviral medication such as neuraminidase
pulmonary complications; sedating medications decrease inhibitors (e.g., oseltamivir phosphate). Early treatment in high-
respiratory effort and should be minimized postoperatively. If risk patients with antiviral medication within 48 hours of onset of
the surgery permits, the use of regional techniques with min- symptoms may reduce morbidity and mortality. Older patients
imal sedation and opioids may be helpful. Good postopera- are less susceptible to the recent pandemic strain, but if infected
tive pulmonary toilet, including deep breathing and coughing, and they develop disease, the clinical manifestations are more
must be encouraged to reduce the risk of postoperative pul- severe. Vaccination is one of the most effective methods to reduce
monary complications. morbidity and mortality associated with influenza. Specific vacci-
nations do not provide protection against other influenza viruses.
The vaccine is effective 14 days after vaccination.
INFECTIOUS DISEASES
Most deaths result from rapidly progressive respiratory fail-
Influenza A ure, ARDS, and refractory shock. These patients deteriorate 3
to 5 days after onset of symptoms. They present with resistant
Although outbreaks of influenza are common, the strain and
hypoxia and frequently require respiratory rescue therapies
severity vary significantly. Influenza pandemics occur every
such as neuromuscular blockade, inhaled NO, prone posi-
few decades and are devastating. More than 40 million people
tioning, and high-frequency oscillatory ventilation (HFOV).
Extracorporeal membrane oxygenation (ECMO) may be
BOX 4-13 n ANESTHESIA CONCERNS FOR PATIENTS beneficial in patients with severe infection, but it is unclear
WITH BLEOMYCIN TOXICITY whether it decreases mortality.
Assess cardiopulmonary function.
Use conservative fluid resuscitation. ANESTHESIA MANAGEMENT
Minimize fraction of inspired oxygen (Fio2). The anesthesiologist is at high risk of exposure to influenza
Ensure aggressive postoperative pulmonary care. virus (Box 4-14). Therefore, full contact precautions are indi-
cated, including disposable fluid-resistant gowns, goggles, face
TABLE 4-9 n Diagnostic Testing for Influenza A
Diagnostic Test Typical Processing Time Sensitivity for H1N1 2009 Method
Rapid influenza diagnostic test 0.5-1 hour 10%-70% Antigen detection

(RIDT)
Direct immunofluorescent assay (DFA) 2-4 hours 47%-93% Antigen detection
rRT-PCR* 48-96 hours 86%-100% RNA detection
Viral culture† 2-10 days ---- Virus isolation
Data from Fartoukh M, Humbert M, Capron F, et al: Severe pulmonary hypertension in histiocytosis X, Am J Respir Crit Care Med 161:216-223, 2000.
*Real-time reverse-transcriptase polymerase chain reaction.
†Differentiate among other influenza A viruses.
Southeast Asia, SARS had made its way to North America dur-
BOX 4-14 n ANESTHESIA CONCERNS FOR PATIENTS ing 2003, with hundreds of cases in Ontario, Canada. More than
WITH INFECTIOUS DISEASE 8000 reported cases of SARS worldwide resulted in over 700
Influenza A (H1N1) deaths. Whether, when, or where another outbreak may occur is
Assess cardiopulmonary function. unknown, but familiarity with the syndrome and how to contain
May have ARDS presentation. the spread are the responsibility of all health care professionals.
Severe cases may require postoperative ventilatory support. Otherwise healthy individuals can be infected by contact
Restrict steroid use.
Anesthesiologist is at high risk of exposure.
or droplet spread, which may be person to person or indi-
Use full contact precautions. rectly through contact with contaminated surfaces, because
Take special care to avoid contamination of equipment and surfaces. the coronavirus can live in the environment for 24 to 48
Provide supportive management. hours. The virus enters the body through mucosal surfaces
Severe Acute Respiratory Syndrome in the respiratory tract and eyes. The incubation period is 2
Assess cardiopulmonary function. to 7 days. Presenting symptoms are vague and include high
Anesthesiologist is at high risk of exposure. fever, dry cough, malaise, myalgia, and shortness of breath,
Use full contact precautions.
which typically progresses to pneumonia and in severe
Take special care to avoid contamination of equipment and surfaces.
Ensure supportive management. cases to v entilator-dependent respiratory distress syndrome.
Diagnosis is based on clinical and epidemiologic data, because
Echinococcal Disease of Lung
Assess pulmonary function.
no laboratory test reliably detects infection early in the clinical
Large cysts may cause respiratory compromise. course.98 Treatment of infected patients is primarily support-
May have one-lung ventilation. ive and similar to that of any other atypical pneumonia. None
Provide supportive management. of the currently available antiviral drugs has been shown to be
effective against SARS-CoV.
ANESTHESIA MANAGEMENT
shields, gloves, and handwashing. Personal protection systems The anesthesiologist's contact with SARS patients occurs pri-
may be advisable for personnel caring for such patients (see marily during airway management for patients in respiratory
SARS). Treatment and perioperative management of those distress (see Box 4-14). The anesthesiologist will be close to
with clinically severe disease is mainly supportive and simi- the patient's upper airway and thus at high risk of exposure to
lar to that of patients with ARDS who require ventilatory the virus. Full contact precautions are recommended, includ-
management. Intraoperative invasive monitors, such as arte- ing disposable fluid-resistant gowns, goggles, face shields,
rial and central catheters, may be useful for cardiopulmonary double gloving, handwashing, and N95 (or equivalent) fit-
management. Corticosteroids should be restricted to patients tested masks.99 Standard surgical face masks and gowns are
with adrenal suppression because these drugs have been asso- inadequate. Removal and disposal of equipment so as not to
ciated with increased mortality in H1N1-infected patients, contaminate the wearer or others is as important as using the
increasing viral shedding time and the risk of coinfection.97 proper protection. Some institutions have taken the added pre-
caution of using a personal protection system (PPS) for per-
sonnel involved in high-risk procedures with SARS patients.
Severe Acute Respiratory Syndrome
These PPS units consist of belt-mounted, powered air puri-
Severe acute respiratory syndrome (SARS) is a highly infectious fiers with high-efficiency particulate air (HEPA) filters and a
disease transmitted by a coronavirus (SARS-CoV). It results lightweight headpiece. Use of this equipment requires training
in atypical pneumonia, which may progress to respiratory as well as adequate donning time. The noise generated by the
distress syndrome. First recognized in 2002 with cases in system makes communication and a uscultation of breath and
cardiac sounds difficult.99,100 Attention must also be directed to is essential to avoid spillage. In the event of contamination,
avoiding contamination of anesthesia workstations and equip- anaphylaxis may occur, and the anesthesiologist should be
ment. This includes placing HEPA filters on the inspiratory prepared by having large-bore IV access, as well as immediate
and expiratory limbs of ventilators and anesthesia machines. availability of epinephrine, diphenhydramine, and corticoste-
Providers must be mindful of everything they touch or that roids (see Box 4-14).
comes into contact with the patient and ensure appropriate
cleaning or disposal of these materials. Maintaining separate
CONCLUSION
clean and dirty work areas may be helpful in this regard.100
Clinical interactions with patients who have uncommon
pulmonary conditions may range from a simple excisional
Echinococcal Disease of Lung
biopsy for asymptomatic pulmonary sarcoid, to a hip replace-
Echinococcal disease or hydatid disease occurs when a human ment in ankylosing spondylitis, to a double-lung transplant
is infected with Echinococcus granulosus, a canine tapeworm. for end-stage cystic fibrosis. The spectrum of procedures
The eggs of the worm are passed in the feces of infested dogs. therefore extends from the routine to the extraordinarily
Humans acquire the infection by unintentionally ingesting complicated. Successful management of patients with respi-
the eggs. Larvae then migrate to the liver, with some eventu- ratory disorders is often challenging in both the conceptual
ally arriving in the lungs and other tissues. The parasites then and technical realms. An understanding of the pathophysi-
mature to form hydatid cysts. The lung forms a protective gran- ology and treatment of the uncommon pulmonary disorder
ulomatous layer around the cyst, which over time becomes will allow the anesthesiologist to anticipate likely clinical
fibrotic. It is estimated that hydatid cysts grow 1 to 2 cm a year.101 problems and tailor anesthetic management to minimize the
As a result of the fecal-oral transmission, echinococcal lung dis- chance of intraoperative and postoperative complications.
ease is more common in children than adults. Overall it is rare
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Respiratory Diseases

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C H A P T E R

ulmonary fibrosis, restrictive lung disease, and pulmonary

complications.2–5 Pre-existing lung disease, smoking, con-

nitrogen (BUN) and creatinine levels will p rovide adequate

TABLE 4-1 n Current Therapies for Primary Pulmonary Hypertension

Therapy Advantages Disadvantages

Phosphodiesterase-5 inhibitors Possible synergy with NO therapy inhibitors —

Endothelin receptor antagonist FDA approval Limited data available

Oxygen Directly reduces pulmonary vascular None

Warfarin (Coumadin) Improved long-term survival; decreases Increased bleeding risk

ECG, Electrocardiographic; Pao2, arterial oxygen tension (partial pressure).

and nitric oxide (NO), alone or in combination, have been

Bronchiolitis Obliterans Organizing Pneumonia Systemic Lupus Erythematosus

with caution because it may divert blood flow from ventilated

hemorrhage, particularly dire manifestations that predict a

be inserted. For procedures involving major fluid shifts,

TABLE 4-9 n Diagnostic Testing for Influenza A

Rapid influenza diagnostic test 0.5-1 hour 10%-70% Antigen detection

Direct immunofluorescent assay (DFA) 2-4 hours 47%-93% Antigen detection

rRT-PCR* 48-96 hours 86%-100% RNA detection

Viral culture† 2-10 days ---- Virus isolation

You might also like

Respiratory Diseases

Uploaded by

Document Information

Original Title

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

Respiratory Diseases

Uploaded by

Copyright:

Available Formats

C H A P T E R

­ ulmonary fibrosis, restrictive lung disease, and pulmonary

complications.2–5 Pre-existing lung disease, smoking, con-

nitrogen (BUN) and creatinine levels will p ­ rovide adequate

TABLE 4-1 n Current Therapies for Primary Pulmonary Hypertension

Therapy Advantages Disadvantages

Phosphodiesterase-5 inhibitors Possible synergy with NO therapy inhibitors —

Endothelin receptor antagonist FDA approval Limited data available

Oxygen Directly reduces pulmonary vascular None

Warfarin (Coumadin) Improved long-term survival; decreases Increased bleeding risk

ECG, Electrocardiographic; Pao2, arterial oxygen tension (partial pressure).

and nitric oxide (NO), alone or in combination, have been

Bronchiolitis Obliterans Organizing Pneumonia Systemic Lupus Erythematosus

with caution because it may divert blood flow from ventilated

hemorrhage, ­particularly dire manifestations that predict a

be inserted. For procedures involving major fluid shifts,

TABLE 4-9 n Diagnostic Testing for Influenza A

Rapid influenza diagnostic test 0.5-1 hour 10%-70% Antigen detection

Direct immunofluorescent assay (DFA) 2-4 hours 47%-93% Antigen detection

rRT-PCR* 48-96 hours 86%-100% RNA detection

Viral culture† 2-10 days ---- Virus isolation

You might also like

ulmonary fibrosis, restrictive lung disease, and pulmonary

nitrogen (BUN) and creatinine levels will p rovide adequate

hemorrhage, particularly dire manifestations that predict a